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FLASH GENE

Symbol

COQ2

contributors: mct/npt/shn - updated : 17-10-2010

HGNC name	coenzyme Q2 homolog, prenyltransferase (yeast)
HGNC id	25223

FLASH GENE DNA RNA EXPRESSION PROTEIN DISORDER ANIMAL & CELL MODELS

Corresponding disease	COQ10D1 coenzyme Q10 deficiency
Location	4q21.23      Physical location : 84.184.978 - 84.206.067
Synonym name	COQ2 homolog
	PHB:polyprenyltransferase
	hCOQ2
	para-hydroxybenzoate--polyprenyltransferase, mitochondrial
	para-hydroxybenzoate-polyprenyltransferase, mitochondrial
Synonym symbol(s)	CL640, FLJ26072, FLJ13014
EC.number	2.5.1.39

DNA

TYPE	functioning gene
STRUCTURE	21.09 kb     7 Exon(s)

MAPPING

cloned

Y

linked

N

status

provisional

Map	cen - D4S400 - D4S2932 - COQ2 - D4S395 - D4S2627 - qter

RNA

TRANSCRIPTS	type	messenger

identification nb exons type bp product Protein kDa AA specific expression Year Pubmed NM_015697 7 - 1657 NP_056512 - 421 - Diomedi-Camassei (2007)

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 Digestive intestine small intestine     highly Endocrine pancreas       highly Reproductive female system uterus cervix   highly

tissue

System Tissue Tissue level 1 Tissue level 2 Level Pubmed Species Stage Rna symbol Blood / hematopoietic bone marrow     highly

cell lineage

cell lines

fluid/secretion

at STAGE

PROTEIN

PHYSICAL PROPERTIES

STRUCTURE

motifs/domains	a central polyprenyl diphosphate-binding site
	6 C-terminal transmembrane regions.

HOMOLOGY

interspecies	ortholog to Coq2, Mus musculus
	ortholog to Coq2, Rattus norvegicus
	ortholog to COQ2, Pan troglodytes

Homologene

FAMILY	ubiA prenyltransferase family
	ubiquinone biosynthetic genes family

CATEGORY

enzyme

SUBCELLULAR LOCALIZATION	plasma membrane
	intracellular
	intracellular,cytoplasm,organelle,mitochondria
	intracellular,cytoplasm,organelle,membrane

basic FUNCTION	involved in the synthesis of CoQ by catalysing the prenylation of p-hydroxybenzoate with an all-trans polyprenyl group
	major function is to transport electrons from complexes I and II to complex III in the respiratory chain which resides in the mitochondrial inner membrane (Quinzii 2008)

CELLULAR PROCESS

PHYSIOLOGICAL PROCESS

mitochondrial transport

text	biosynthesis of ubiquinone

PATHWAY

metabolism

signaling

part of the CoQ(10) biosynthetic pathways with ADCK3, PDSS2, PDSS1, COQ6, COQ9

a component

INTERACTION

DNA

RNA

small molecule

protein

cell & other

REGULATION

ASSOCIATED DISORDERS

corresponding disease(s)

COQ10D1

Susceptibility

Variant & Polymorphism

Candidate gene
Marker
Therapy target	CoQ10 administration

ANIMAL & CELL MODELS