Browsing Compounds

CL(i-14:0/i-16:0/i-24:0/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-16:0/i-24:0/i-20:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isohexadecanoic acid at the C-2 position, one chain of isotetracosanoic 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).

CL(i-14:0/i-16:0/i-24:0/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-16:0/i-24:0/i-21:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isohexadecanoic acid at the C-2 position, one chain of isotetracosanoic 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).

CL(i-14:0/i-16:0/i-24:0/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-16:0/i-24:0/i-22:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isohexadecanoic acid at the C-2 position, one chain of isotetracosanoic 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).

CL(i-14:0/i-16:0/i-24:0/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-16:0/i-24:0/i-24:0), in particular, consists of one chain of isotetradecanoic acid at the C-1 position, one chain of isohexadecanoic acid at the C-2 position, one chain of isotetracosanoic 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).

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

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

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

CL(i-14:0/i-17:0/a-13:0/18:2(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-13:0/18:2(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 anteisotridecanoic 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).

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

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

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

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

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

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

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

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

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

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

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

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