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Showing 55521 - 55540 of 55723 compounds

Compound ID

Compound

Pathways

PW_C096597

Image HMDB0092879: View Metabocard

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

CL(i-14:0/i-17:0/a-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/a-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 anteisoheptadecanoic 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_C096598

Image HMDB0092896: View Metabocard

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

CL(i-14:0/i-17:0/a-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/a-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 anteisoheptadecanoic 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_C096599

Image HMDB0092891: View Metabocard

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

CL(i-14:0/i-17:0/a-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/a-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 anteisoheptadecanoic 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_C096600

Image HMDB0092875: View Metabocard

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

CL(i-14:0/i-17:0/a-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/a-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 anteisoheptadecanoic 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_C096601

Image HMDB0092816: View Metabocard

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

CL(i-14:0/i-17:0/a-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/a-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 anteisoheptadecanoic 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_C096602

Image HMDB0092880: View Metabocard

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

CL(i-14:0/i-17:0/a-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/a-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 anteisoheptadecanoic 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_C096603

Image HMDB0092898: View Metabocard

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

CL(i-14:0/i-17:0/a-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/a-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 anteisoheptadecanoic 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_C096604

Image HMDB0092813: View Metabocard

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

CL(i-14:0/i-17:0/a-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/a-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 anteisoheptadecanoic 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_C096605

Image HMDB0092829: View Metabocard

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

CL(i-14:0/i-17:0/a-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/a-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 anteisoheptadecanoic 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_C096606

Image HMDB0092874: View Metabocard

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

CL(i-14:0/i-17:0/a-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/a-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 anteisoheptadecanoic 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_C096607

Image HMDB0092801: View Metabocard

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

CL(i-14:0/i-17:0/a-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/a-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 anteisoheptadecanoic 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_C096608

Image HMDB0092842: View Metabocard

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

CL(i-14:0/i-17:0/a-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/a-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 anteisoheptadecanoic 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_C096609

Image HMDB0092833: View Metabocard

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

CL(i-14:0/i-17:0/a-17:0/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-17:0/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 anteisoheptadecanoic 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_C096610

Image HMDB0092856: View Metabocard

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

CL(i-14:0/i-17:0/a-17:0/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-17:0/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 anteisoheptadecanoic 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_C096611

Image HMDB0092868: View Metabocard

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

CL(i-14:0/i-17:0/a-17:0/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-17:0/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 anteisoheptadecanoic 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_C096612

Image HMDB0092806: View Metabocard

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

CL(i-14:0/i-17:0/a-17:0/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-17:0/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 anteisoheptadecanoic 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_C096613

Image HMDB0092882: View Metabocard

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

CL(i-14:0/i-17:0/a-17:0/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-17:0/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 anteisoheptadecanoic 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_C096614

Image HMDB0092582: View Metabocard

CL(i-14:0/i-17:0/i-12:0/18:2(9Z,11Z))

CL(i-14:0/i-17:0/i-12:0/18:2(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-12:0/18:2(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 isododecanoic 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_C096615

Image HMDB0092569: View Metabocard

CL(i-14:0/i-17:0/i-12:0/a-13:0)

CL(i-14:0/i-17:0/i-12:0/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-12:0/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 isododecanoic 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_C096616

Image HMDB0092575: View Metabocard

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

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