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FLASH GENE

Symbol

COX10

contributors: shn - updated : 15-10-2010

HGNC name	COX10 homolog, cytochrome c oxidase assembly protein, heme A: farnesyltransferase (yeast)
HGNC id	2260

FLASH GENE DNA RNA EXPRESSION PROTEIN DISORDER ANIMAL & CELL MODELS

EXPRESSION

Type

widely

expressed in

(based on citations)

organ(s)

System Organ level 1 Organ level 2 Organ level 3 Organ level 4 Level Pubmed Species Stage Rna symbol Cardiovascular heart       highly Digestive intestine small intestine     lowly   intestine large intestine colon   moderately   liver       lowly Endocrine pancreas       lowly Lymphoid/Immune spleen       lowly   thymus       lowly Nervous brain       lowly Reproductive female system placenta     lowly   female system ovary     lowly   male system prostate     lowly   male system testis     highly Respiratory lung       lowly Urinary kidney       lowly

tissue

System Tissue Tissue level 1 Tissue level 2 Level Pubmed Species Stage Rna symbol Muscular striatum skeletal   highly

cells

System Cell Pubmed Species Stage Rna symbol Blood/Hematopoietic leukocyte

cell lineage

cell lines

fluid/secretion

at STAGE

PROTEIN

PHYSICAL PROPERTIES

STRUCTURE

motifs/domains	a long relatively hydrophilic N terminal
	seve to nine potential transmembrane segments (TM9)

mono polymer

homomer , complex

HOMOLOGY

interspecies	ortholog to COX10, S.cerevisiae
	ortholog to COX10, Pan troglodytes
	ortholog to Cox10, Mus musculus
	ortholog to cox10, Danio rerio

Homologene

FAMILY

ub1a prenyltransferase family

CATEGORY

enzyme , transport

SUBCELLULAR LOCALIZATION	intracellular
	intracellular,cytoplasm,organelle,mitochondria,inner
	intracellular,cytoplasm,organelle,membrane
text	localized in mitochondrial inner membrane

basic FUNCTION	terminal component of the mitochondrial respiratory chain that catalyzes the electron transfer from reduced cytochrome c to oxygen
	catalyzes the conversion of protoheme (heme B) to heme O via the farnesylation of a vinyl group at position C2 in the first step of the mitochondrial heme A biosynthetic
	involved in heme A biosynthesis

CELLULAR PROCESS

PHYSIOLOGICAL PROCESS

mitochondrial transport , electron transport

text	cellular respiration

PATHWAY

metabolism

energetic

signaling

a component

INTERACTION

DNA

RNA

small molecule

protein

ISCU (iron-sulfur cluster scaffold homolog) and COX10 (cytochrome c oxidase assembly protein), two important factors of the mitochondria electron transport chain and the tricarboxylic acid cycle have been identified as potential targets of MIR210

cell & other

REGULATION

ASSOCIATED DISORDERS

corresponding disease(s)

COX10D

Other morbid association(s)

Type Gene Modification Chromosome rearrangement Protein expression Protein Function constitutional   deletion     in HNPP (hereditary neuropathy with liability to pressure palsies)

Susceptibility

Variant & Polymorphism

Candidate gene

Marker

Therapy target

ANIMAL & CELL MODELS

	COX10-null mice have only 20-40% of wild-type mitochondrial function and the transgenic overexpressionof PGC-1a in these mice restores full mitochondrial function
	COX10-/- mice treated with bezafibrate have 85% of WT OxPhos activity and their muscle function is improved compared with untreated littermates