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FLASH GENE

Symbol

EHHADH

contributors: - updated : 13-10-2006

HGNC name	enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase
HGNC id	3247

FLASH GENE DNA RNA EXPRESSION PROTEIN DISORDER ANIMAL & CELL MODELS

Corresponding disease	PBE Zellweger-like syndrome
Location	3q26.3-q28      Physical location : 184.908.413 - 184.971.886
Synonym name	L-bifunctional protein,peroxisomal
Synonym symbol(s)	LBP, LBFP, MGC120586, ECHD
EC.number	4.2.1.17, 1.1.1.35

DNA

TYPE	functioning gene
STRUCTURE	63.00 kb     7 Exon(s)

10 Kb 5' upstream gene genomic sequence study

MAPPING

cloned

Y

linked

N

status

provisional

RNA

TRANSCRIPTS	type	messenger

EXPRESSION

Type

constitutive of

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 liver       highly Nervous brain       moderately Reproductive male system prostate     moderately Respiratory lung       moderately Urinary kidney       highly

cell lineage

cell lines

fluid/secretion

at STAGE

PROTEIN

PHYSICAL PROPERTIES

STRUCTURE

motifs/domains	N terminal dehydrogenase activity
	two hydratase activities in the central part
	a C terminal region of strong homology with sterol carrier protein

mono polymer

monomer

HOMOLOGY

Homologene

FAMILY

CATEGORY

enzyme

SUBCELLULAR LOCALIZATION	intracellular
	intracellular,cytoplasm,organelle,peroxisome

basic FUNCTION	bifunctional enzyme (matrix) including enoyl-CoA hydratase (3-hydroxyacyl-CoA-dehydratase) and 3-hydroxyacyl-CoA dehydrogenase
	catalyzing the second and third step of peroxisomal fatty acid beta-oxidation of very long chain fatty acids, but not bile intermediates

CELLULAR PROCESS

PHYSIOLOGICAL PROCESS

text	peroxisomal beta-oxidation of very long chain fatty acids

PATHWAY

metabolism

signaling

a component

INTERACTION

DNA

RNA

small molecule

protein

cell & other

REGULATION

ASSOCIATED DISORDERS

corresponding disease(s)

PBE

Susceptibility

Variant & Polymorphism

Candidate gene

Marker

Therapy target

ANIMAL & CELL MODELS