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Showing 55501 - 55520 of 55723 compounds

Compound ID

Compound

Pathways

PW_C096577

Image HMDB0092665: View Metabocard

CL(i-14:0/i-17:0/a-13:0/i-24:0)

CL(i-14:0/i-17:0/a-13:0/i-24:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-13:0/i-24:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisotridecanoic acid at the C-3 position, and one chain of isotetracosanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096578

Image HMDB0092748: View Metabocard

CL(i-14:0/i-17:0/a-15:0/18:2(9Z,11Z))

CL(i-14:0/i-17:0/a-15:0/18:2(9Z,11Z)) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/18:2(9Z,11Z)), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of (9Z,11Z)-octadecadienoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096579

Image HMDB0092716: View Metabocard

CL(i-14:0/i-17:0/a-15:0/a-13:0)

CL(i-14:0/i-17:0/a-15:0/a-13:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/a-13:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of anteisotridecanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096580

Image HMDB0092728: View Metabocard

CL(i-14:0/i-17:0/a-15:0/a-15:0)

CL(i-14:0/i-17:0/a-15:0/a-15:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/a-15:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of anteisopentadecanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096581

Image HMDB0092740: View Metabocard

CL(i-14:0/i-17:0/a-15:0/a-17:0)

CL(i-14:0/i-17:0/a-15:0/a-17:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/a-17:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of anteisoheptadecanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096582

Image HMDB0092764: View Metabocard

CL(i-14:0/i-17:0/a-15:0/a-21:0)

CL(i-14:0/i-17:0/a-15:0/a-21:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/a-21:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of anteisoheneicosanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096583

Image HMDB0092778: View Metabocard

CL(i-14:0/i-17:0/a-15:0/a-25:0)

CL(i-14:0/i-17:0/a-15:0/a-25:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/a-25:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of anteisopentacosanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096584

Image HMDB0092709: View Metabocard

CL(i-14:0/i-17:0/a-15:0/i-12:0)

CL(i-14:0/i-17:0/a-15:0/i-12:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/i-12:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of isododecanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096585

Image HMDB0092714: View Metabocard

CL(i-14:0/i-17:0/a-15:0/i-13:0)

CL(i-14:0/i-17:0/a-15:0/i-13:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/i-13:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of isotridecanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096586

Image HMDB0092721: View Metabocard

CL(i-14:0/i-17:0/a-15:0/i-14:0)

CL(i-14:0/i-17:0/a-15:0/i-14:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/i-14:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of isotetradecanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096587

Image HMDB0092726: View Metabocard

CL(i-14:0/i-17:0/a-15:0/i-15:0)

CL(i-14:0/i-17:0/a-15:0/i-15:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/i-15:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of isopentadecanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096588

Image HMDB0092733: View Metabocard

CL(i-14:0/i-17:0/a-15:0/i-16:0)

CL(i-14:0/i-17:0/a-15:0/i-16:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/i-16:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of isohexadecanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096589

Image HMDB0092738: View Metabocard

CL(i-14:0/i-17:0/a-15:0/i-17:0)

CL(i-14:0/i-17:0/a-15:0/i-17:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/i-17:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of isoheptadecanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096590

Image HMDB0092747: View Metabocard

CL(i-14:0/i-17:0/a-15:0/i-18:0)

CL(i-14:0/i-17:0/a-15:0/i-18:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/i-18:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of isooctadecanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096591

Image HMDB0092753: View Metabocard

CL(i-14:0/i-17:0/a-15:0/i-19:0)

CL(i-14:0/i-17:0/a-15:0/i-19:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/i-19:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of isononadecanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096592

Image HMDB0092757: View Metabocard

CL(i-14:0/i-17:0/a-15:0/i-20:0)

CL(i-14:0/i-17:0/a-15:0/i-20:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/i-20:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of isoeicosanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096593

Image HMDB0092762: View Metabocard

CL(i-14:0/i-17:0/a-15:0/i-21:0)

CL(i-14:0/i-17:0/a-15:0/i-21:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/i-21:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of isoheneicosanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096594

Image HMDB0092769: View Metabocard

CL(i-14:0/i-17:0/a-15:0/i-22:0)

CL(i-14:0/i-17:0/a-15:0/i-22:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/i-22:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of isodocosanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096595

Image HMDB0092773: View Metabocard

CL(i-14:0/i-17:0/a-15:0/i-24:0)

CL(i-14:0/i-17:0/a-15:0/i-24:0) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-15:0/i-24:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisopentadecanoic acid at the C-3 position, and one chain of isotetracosanoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).

PW_C096596

Image HMDB0092834: View Metabocard

CL(i-14:0/i-17:0/a-17:0/18:2(9Z,11Z))

CL(i-14:0/i-17:0/a-17:0/18:2(9Z,11Z)) is a cardiolipin (CL). Cardiolipins (bisphosphatidyl glycerol) are an important component of the inner mitochondrial membrane, where they constitute about 20% of the total lipid. Cardiolipins are a "double" phospholipid because they have four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized on the matrix side of the inner mitochondrial membrane. They are highly abundant in metabolically active cells (heart, muscle) and play an important role in the blood clotting process. CL(i-14:0/i-17:0/a-17:0/18:2(9Z,11Z)), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isoheptadecanoic acid at the C-2 position, one chain of anteisoheptadecanoic acid at the C-3 position, and one chain of (9Z,11Z)-octadecadienoic acid at the C-4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. Newly synthesized cardiolipins undergo remodeling, a process carried out by the enzyme tafazzin. A mutated tafazzin gene disrupts this post-synthetic remodeling and causes Barth syndrome (BTHS), an X-linked human disease (PMID: 16973164). BTHS patients seem to lack acyl specificity and consequently, many potential cardiolipin species can exist (PMID: 16226238).
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