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Cardiolipin (CL) is considered the signature phospholipid of the inner mitochondrial membrane (IMM) which is also the site of its synthesis. Structurally, CL is a lipid dimer with two phosphatidyl groups connected by a glycerol backbone. It has two chiral carbons and four fatty acyl chains, usually polyunsaturated. It can interact with various proteins that include components of the electron transport chain (ETC), enzymes and metabolite carriers. CL is found in the crystal structures of complex

Pathway Diagram:

Elsevier Inc. cardiolipin --+> metabolite exchange ATP synthase (complex V) Ckmt1 ATP/ADP exchange ATP biosynthetic pathway respiratory complex IV respiratory complex III respiratory complex I phosphatidylglycerol ---> cardiolipin phosphatidic acid ---> CDP-diacylglycerol cytidine triphosphate ---> CDP-diacylglycerol CDP-diacylglycerol cardiolipin ---- CKMT1B cardiolipin ---> translocated cardiolipin CKMT1B ---- translocated cardiolipin Plscr3 ---- translocated cardiolipin Ckmt1 ---- translocated cardiolipin Nme4 ---- translocated cardiolipin cardiolipin ---- Nme4 CDP-diacylglycerol ---> phosphatidylglycerophosphate phosphatidylglycerophosphate ---> phosphatidylglycerol sustained mitochondrial injury translocated cardiolipin peroxidized cardiolipin Tamm41 Pgs1 Ptpmt1 Crls1 Taz CKMT1B Nme4 Plscr3 Cycs ---- translocated cardiolipin Cycs ---> peroxidized cardiolipin Cycs --+> intrinsic apoptotic pathway peroxidized cardiolipin --+> intrinsic apoptotic pathway phosphatidylglycerophosphate phosphatidylglycerol cytidine triphosphate phosphatidic acid mitochondrial autophagy pathway intrinsic apoptotic pathway mitochondrial injury Cycs cardiolipin metabolite exchange electron transport chain pathway cardiolipin ---- Ckmt1 cardiolipin --+> ATP biosynthetic pathway cardiolipin --+> electron transport chain pathway translocated cardiolipin --+> mitochondrial autophagy pathway Taz ---> cardiolipin

Genes in Pathway:

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cardiolipin metabolic pathway term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Ckmt1 creatine kinase, mitochondrial 1 JBrowse link 3 113,318,559 113,324,459 RGD:10400850
G Cycs cytochrome c, somatic JBrowse link 4 80,331,226 80,333,326 RGD:10400850
G Nme4 NME/NM23 nucleoside diphosphate kinase 4 JBrowse link 10 15,461,567 15,465,422 RGD:10400850
G Plscr3 phospholipid scramblase 3 JBrowse link 10 56,452,632 56,458,390 RGD:10400850
G Taz tafazzin JBrowse link X 156,421,006 156,429,461 RGD:10400850
cardiolipin biosynthetic pathway term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Crls1 cardiolipin synthase 1 JBrowse link 3 125,503,638 125,522,460 RGD:10400850
G Pgs1 phosphatidylglycerophosphate synthase 1 JBrowse link 10 106,994,595 107,030,208 RGD:10400850
G Ptpmt1 protein tyrosine phosphatase, mitochondrial 1 JBrowse link 3 79,734,961 79,743,737 RGD:10400850
G Tamm41 TAM41 mitochondrial translocator assembly and maintenance homolog JBrowse link 4 146,922,085 146,954,159 RGD:10400850

Pathway Gene Annotations

Disease Annotations Associated with Genes in the cardiolipin metabolic pathway
Pathway Annotations Associated with Genes in the cardiolipin metabolic pathway

References Associated with the cardiolipin metabolic pathway:

Ontology Path Diagram:

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