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FLASH GENE

Symbol

SURF1

contributors: mct/ - updated : 05-12-2016

HGNC name	surfeit 1
HGNC id	11474

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 Digestive liver         Endocrine pancreas

tissue

System Tissue Tissue level 1 Tissue level 2 Level Pubmed Species Stage Rna symbol Connective bone

cell lineage

cell lines

fluid/secretion

at STAGE

physiological period

PROTEIN

PHYSICAL PROPERTIES

STRUCTURE

motifs/domains	an aminoterminal characteristic mitochondrial targeting sequence
	N and C termini transmembrane segments
	a central loop spanning the intermembrane space

mono polymer

heteromer , complex

HOMOLOGY

interspecies	homolog to yeast SHY1(mitochondrial protein)
	ortholog to murine Surf1
	homolog to Drosophila Surf1
	homolog to C.elegans H06I04.2

Homologene

FAMILY

SURF1 family

CATEGORY

enzyme , transport carrier

SUBCELLULAR LOCALIZATION	intracellular
	intracellular,cytoplasm,organelle,mitochondria,inner
	intracellular,cytoplasm,organelle,membrane
text	anchored to the inner mitochondrial membrane by two transmembrane domains

basic FUNCTION	involved in the biogenesis of an active cytochrome c oxidase complex
	having a direct role in heme a cofactor insertion into COX subunit I by providing a protein-bound heme a pool
	appears to be involved in the early step of COX assembly

CELLULAR PROCESS

PHYSIOLOGICAL PROCESS

respiration , electron transport

text	aerobic respiration

PATHWAY

metabolism

signaling

a component

associated with a primitive quinol oxidase belonging to the cytochrome oxidase

INTERACTION

DNA

RNA

small molecule

protein

is a specific assembly factor of cytochrome c oxidase (COX)

cell & other

REGULATION

ASSOCIATED DISORDERS

corresponding disease(s)

SNELS , CMT4K

related resource

MITOP database

Susceptibility

Variant & Polymorphism

Candidate gene

Marker

Therapy target

ANIMAL & CELL MODELS

	SURF1(-/-) mouse fibroblasts were characterized by low content of COX1 assembly intermediates and milder decrease in COX monomer, which appeared more stable
	mice carrying a homozygous knockout (KO) of the Surf1 gene showed a significant decrease in mitochondrial electron transport chain Complex IV activity, yet displayed increased lifespan and reduced brain damage after excitotoxic insults