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FLASH GENE

Symbol

TIMM22

contributors: mct - updated : 23-05-2019

HGNC name	translocase of inner mitochondrial membrane 22 homolog (yeast)
HGNC id	17317

FLASH GENE DNA RNA EXPRESSION PROTEIN DISORDER ANIMAL & CELL MODELS

Corresponding disease	TIMM22D TIMM22 deficiency
Location	17p13.3      Physical location : 900.356 - 905.388
Synonym name	testis-expressed sequence 4
	mitochondrial import inner membrane translocase subunit
	putative membrane protein
Synonym symbol(s)	TIM22, TEX4

DNA

TYPE	functioning gene
STRUCTURE	5.03 kb     4 Exon(s)

10 Kb 5' upstream gene genomic sequence study

MAPPING

cloned

Y

linked

N

status

provisional

RNA

TRANSCRIPTS	type	messenger

identification nb exons type bp product Protein kDa AA specific expression Year Pubmed NM_013337 4 - 1691 NP_037469 20 194 - -

EXPRESSION

Type

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         Digestive liver           stomach       highly Endocrine pancreas         Nervous brain         Reproductive female system ovary     predominantly   male system prostate         male system testis       Respiratory lung       moderately Urinary kidney

tissue

System Tissue Tissue level 1 Tissue level 2 Level Pubmed Species Stage Rna symbol Blood / hematopoietic bone marrow       Epithelial secretory glandular endocrine   Epithelial secretory glandular exocrine

cell lineage
cell lines
fluid/secretion	blood

at STAGE

PROTEIN

PHYSICAL PROPERTIES		Hydrophilic
STRUCTURE

motifs/domains

HOMOLOGY

interspecies	homolog to rattus Timm22 (94.2 pc)
	homolog to murine Timm22 (95.4 pc)

Homologene

FAMILY

TIM17/TIM22/TIM23 family

CATEGORY

enzyme , transport carrier

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

basic FUNCTION	mediating the import and insertion of multi-pass transmembrane proteins into the mitochondrial inner membrane
	constituting the voltage-activated and signal-gated channel, in the TIM22 complex
	forming a twin-pore translocase that uses the membrane potential as external driving force in 2 voltage-dependent steps
	majority of inner membrane protein precursors are synthesized in the cytosol, but precursors with a cleavable presequence are imported by the presequence translocase (TIMM23 complex), while other precursors containing internal targeting signals are imported by the carrier translocase (TIMM22 complex)
	the carrier translocase (TIMM22 complex) inserts multispanning proteins into the inner membrane
	TIMM22, TIMM23 import pathways depend on the presence of a membrane potential, which is generated by the activity of the respiratory chain
	TIMM22 complex mediates the import of hydrophobic carrier proteins into the mitochondrial inner membrane

CELLULAR PROCESS

PHYSIOLOGICAL PROCESS

mitochondrial transport

text	protein transport

PATHWAY

metabolism

signaling

TIM22 protein import pathway mediates the import of membrane proteins into the mitochondrial inner membrane (Hasson 2010)

a component

TIM22 protein import pathway consists of two intermembrane space chaperone complexes, the TIMM9-TIMM10 and TIMM8A-TIMM13 complexes (Hasson 2010)

INTERACTION

DNA

RNA

small molecule

protein	interacting directly with TIMM9, TIMM10/TIM10A and FXC1/TIM10B
	TIMM29 is required for the stability of the TIMM22 complex and functions in the assembly of TIMM22
	TIMM29 is required for the structural integrity of the TIMM22 complex and for import of substrate proteins by the carrier translocase
	AGK functions in a kinase-independent manner to maintain the integrity of the TIMM22 complex, where it facilitates the import and assembly of mitochondrial carrier proteins
	VDAC1 interacts with carrier precursors arriving in the intermembrane space and recruits TIMM22 complexes, thus ensuring an efficient transfer of the precursors to the inner membrane translocase

cell & other

REGULATION

ASSOCIATED DISORDERS

corresponding disease(s)

TIMM22D

Susceptibility

Variant & Polymorphism

Candidate gene

Marker

Therapy target

ANIMAL & CELL MODELS