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Showing 55621 - 55640 of 55723 compounds

Compound ID

Compound

Pathways

PW_C096697

Image HMDB0092795: View Metabocard

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

CL(i-14:0/i-17:0/i-16:0/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/i-16:0/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 isohexadecanoic 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_C096698

Image HMDB0092836: View Metabocard

CL(i-14:0/i-17:0/i-16:0/i-18:0)

CL(i-14:0/i-17:0/i-16:0/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/i-16:0/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 isohexadecanoic 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_C096699

Image HMDB0092822: View Metabocard

CL(i-14:0/i-17:0/i-16:0/i-19:0)

CL(i-14:0/i-17:0/i-16:0/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/i-16:0/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 isohexadecanoic 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_C096700

Image HMDB0092814: View Metabocard

CL(i-14:0/i-17:0/i-16:0/i-20:0)

CL(i-14:0/i-17:0/i-16:0/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/i-16:0/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 isohexadecanoic 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_C096701

Image HMDB0092812: View Metabocard

CL(i-14:0/i-17:0/i-16:0/i-21:0)

CL(i-14:0/i-17:0/i-16:0/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/i-16:0/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 isohexadecanoic 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_C096702

Image HMDB0092808: View Metabocard

CL(i-14:0/i-17:0/i-16:0/i-22:0)

CL(i-14:0/i-17:0/i-16:0/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/i-16:0/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 isohexadecanoic 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_C096703

Image HMDB0092831: View Metabocard

CL(i-14:0/i-17:0/i-16:0/i-24:0)

CL(i-14:0/i-17:0/i-16:0/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/i-16:0/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 isohexadecanoic 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_C096704

Image HMDB0092844: View Metabocard

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

CL(i-14:0/i-17:0/i-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/i-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 isoheptadecanoic 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_C096705

Image HMDB0092852: View Metabocard

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

CL(i-14:0/i-17:0/i-17:0/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/i-17:0/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 isoheptadecanoic 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_C096706

Image HMDB0092851: View Metabocard

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

CL(i-14:0/i-17:0/i-17:0/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/i-17:0/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 isoheptadecanoic 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_C096707

Image HMDB0092865: View Metabocard

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

CL(i-14:0/i-17:0/i-17:0/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/i-17:0/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 isoheptadecanoic 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_C096708

Image HMDB0092820: View Metabocard

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

CL(i-14:0/i-17:0/i-17:0/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/i-17:0/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 isoheptadecanoic 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_C096709

Image HMDB0092843: View Metabocard

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

CL(i-14:0/i-17:0/i-17:0/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/i-17:0/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 isoheptadecanoic 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_C096710

Image HMDB0092802: View Metabocard

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

CL(i-14:0/i-17:0/i-17:0/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/i-17:0/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 isoheptadecanoic 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_C096711

Image HMDB0092858: View Metabocard

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

CL(i-14:0/i-17:0/i-17:0/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/i-17:0/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 isoheptadecanoic 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_C096712

Image HMDB0092824: View Metabocard

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

CL(i-14:0/i-17:0/i-17:0/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/i-17:0/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 isoheptadecanoic 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_C096713

Image HMDB0092807: View Metabocard

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

CL(i-14:0/i-17:0/i-17:0/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/i-17:0/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 isoheptadecanoic 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_C096714

Image HMDB0092825: View Metabocard

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

CL(i-14:0/i-17:0/i-17:0/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/i-17:0/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 isoheptadecanoic 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_C096715

Image HMDB0092839: View Metabocard

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

CL(i-14:0/i-17:0/i-17:0/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/i-17:0/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 isoheptadecanoic 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_C096716

Image HMDB0092838: View Metabocard

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

CL(i-14:0/i-17:0/i-17:0/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/i-17:0/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 isoheptadecanoic 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).
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