Mtch2 mitochondrial carrier 2 [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Mtch2provided by MGI
Official Full Name: mitochondrial carrier 2provided by MGI
Primary source: MGI:MGI:1929260
See related: Ensembl:ENSMUSG00000027282 AllianceGenome:MGI:1929260
Gene type: protein coding
RefSeq status: REVIEWED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: HSPC032; 2310034D24Rik; 4930539J07Rik
Summary: This gene encodes a member of the SLC25 family of nuclear-encoded transporters that are localized in the inner mitochondrial membrane. Members of this superfamily are involved in many metabolic pathways and cell functions. Genome-wide association studies in human have identified single-nucleotide polymorphisms in several loci associated with obesity. This gene is one such locus, which is highly expressed in white adipose tissue and adipocytes, and thought to play a regulatory role in adipocyte differentiation and biology. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. A recent study showed this gene to be an authentic stop codon readthrough target that can produce two isoforms from the same mRNA by use of alternative in-frame translation termination codons. [provided by RefSeq, Dec 2017]
Expression: Ubiquitous expression in testis adult (RPKM 96.2), large intestine adult (RPKM 54.2) and 27 other tissues See more
Orthologs: human all
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Genomic context

Location:: 2 E1; 2 50.41 cM

Exon count:: 13

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	2	NC_000068.8 (90677434..90697154)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	2	NC_000068.7 (90847090..90866810)

Chromosome 2 - NC_000068.8 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:

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Expression

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See details

Project title: Mouse ENCODE transcriptome data
Description: RNA profiling data sets generated by the Mouse ENCODE project.
BioProject: PRJNA66167
Publication: PMID 25409824
Analysis date: n/a

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Silica nanoparticles inducing the apoptosis via microRNA-450b-3p targeting MTCH2 in mice and spermatocyte cell.
Title: Silica nanoparticles inducing the apoptosis via microRNA-450b-3p targeting MTCH2 in mice and spermatocyte cell.
Downregulated miR-150 in bone marrow mesenchymal stem cells attenuates the apoptosis of LPS-stimulated RAW264.7 via MTCH2-dependent mitochondria transfer.
Title: Downregulated miR-150 in bone marrow mesenchymal stem cells attenuates the apoptosis of LPS-stimulated RAW264.7 via MTCH2-dependent mitochondria transfer.
Mitochondrial Carrier Homolog 2 forebrain conditional knockout mice exhibit impaired spatial but not motor learning and an impairment in long-term potentiation in hippocampal slices.
Title: Learning Deficits in Adult Mitochondria Carrier Homolog 2 Forebrain Knockout Mouse.
Mitochondrial carrier homolog 2 protein (MTCH2) knockout (-/-) embryonic stem cells (ESCs) fail to elongate their mitochondria and to alter their metabolism.
Title: MTCH2-mediated mitochondrial fusion drives exit from naïve pluripotency in embryonic stem cells.
MTCH2 is a critical player in neuronal cell biology, controlling mitochondria metabolism, motility and calcium buffering to regulate hippocampal-dependent cognitive functions.
Title: Loss of forebrain MTCH2 decreases mitochondria motility and calcium handling and impairs hippocampal-dependent cognitive functions.
Results show that Mimp/Mtch2 overexpression alters lipid metabolism and may play a role in the onset of obesity and development of insulin resistance.
Title: Mimp/Mtch2, an Obesity Susceptibility Gene, Induces Alteration of Fatty Acid Metabolism in Transgenic Mice.
MTCH2 is a conserved regulator of lipid homeostasis. MTCH2 was found to be both required and sufficient for lipid homeostasis shifts, suggesting that pharmacological inhibition of MTCH2 could be therapeutic for treatment of obesity and related disorders. MTCH2 could influence lipid homeostasis through inhibition of ESR1 activity.
Title: MTCH2 is a conserved regulator of lipid homeostasis.
Loss of MTCH2 increases muscle metabolism, energy expenditure, heart function, and protects from diet-induced obesity.
Title: Loss of Muscle MTCH2 Increases Whole-Body Energy Utilization and Protects from Diet-Induced Obesity.
Loss of Mtch2 increases mitochondrial OXPHOS, triggering HSC entry into cycle. Elevated OXPHOS is accompanied by an increase in mitochondrial size, increase in ATP and ROS levels, and protection from irradiation-induced apoptosis.
Title: An MTCH2 pathway repressing mitochondria metabolism regulates haematopoietic stem cell fate.
Data indicate that Mtch2 accelerated conformational change in membrane-bound tBid (Bid) enables it to activate Bax.
Title: tBid undergoes multiple conformational changes at the membrane required for Bax activation.

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Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Gene trapped (1)
Targeted (5) 1 citation
Endonuclease-mediated (3)

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

D9Mit34.1 (e-PCR), detects polymorphism
- UniSTS:131903

NoName (e-PCR)
- UniSTS:234450

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables membrane insertase activity	ISO Inferred from Sequence Orthology more info
enables protein binding	IPI Inferred from Physical Interaction more info	PubMed

Process	Evidence Code	Pubs
acts_upstream_of_or_within apoptotic process	IEA Inferred from Electronic Annotation more info
acts_upstream_of_or_within cellular response to radiation	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within establishment of protein localization to mitochondrial membrane involved in mitochondrial fission	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within hematopoietic stem cell homeostasis	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within hematopoietic stem cell migration	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within hepatocyte apoptotic process	IGI Inferred from Genetic Interaction more info	PubMed
acts_upstream_of_or_within lactate metabolic process	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in lipid homeostasis	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within mitochondrial ATP synthesis coupled electron transport	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within negative regulation of glycolytic process	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within negative regulation of mitochondrial membrane potential	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in positive regulation of apoptotic process	IBA Inferred from Biological aspect of Ancestor more info
involved_in positive regulation of apoptotic process	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within positive regulation of cysteine-type endopeptidase activity involved in apoptotic process	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within positive regulation of intrinsic apoptotic signaling pathway in response to DNA damage	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in positive regulation of stem cell differentiation	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in protein insertion into mitochondrial outer membrane	ISO Inferred from Sequence Orthology more info
acts_upstream_of_or_within protein localization to mitochondrion	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within protein localization to mitochondrion	ISO Inferred from Sequence Orthology more info
involved_in regulation of mitochondrial fusion	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within regulation of mitochondrial membrane permeability involved in apoptotic process	IGI Inferred from Genetic Interaction more info	PubMed

Component	Evidence Code	Pubs
is_active_in membrane	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrial inner membrane	HDA more info	PubMed
located_in mitochondrial outer membrane	IDA Inferred from Direct Assay more info	PubMed
is_active_in mitochondrial outer membrane	ISO Inferred from Sequence Orthology more info
located_in mitochondrion	HDA more info	PubMed
is_active_in mitochondrion	IBA Inferred from Biological aspect of Ancestor more info

General protein information

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Preferred Names: mitochondrial carrier homolog 2

Names: met-induced mitochondrial protein

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

NM_001317241.1 → NP_001304170.1 mitochondrial carrier homolog 2 isoform 1x

Status: REVIEWED

Description

Transcript Variant: This variant (1) represents the predominant transcript and encodes two isoforms, which result from the use of alternative in-frame translation termination codons. The shorter isoform (1) results from translation termination at the upstream UGA stop codon, while the longer isoform (1x) results from UGA stop codon readthrough to the downstream UAG termination codon. This RefSeq represents the longer, C-terminally extended isoform (1x). As the UGA stop codon has been reported to specify several alternative amino acids (tryptophan, cysteine, arginine and serine), its location in the longer isoform is denoted by an 'X'.

Source sequence(s)

AK008712, AK164169

UniProtKB/TrEMBL

Q3UA17

Conserved Domains (1) summary

pfam00153
Location:128 → 192

Mito_carr; Mitochondrial carrier protein
NM_001317242.1 → NP_001304171.1 mitochondrial carrier homolog 2 isoform 2

Status: REVIEWED

Description

Transcript Variant: This variant (2) uses an alternate in-frame acceptor splice site at an internal coding exon compared to variant 1. The resulting isoform (2) is longer containing a 9 aa protein segment not found in isoform 1.

Source sequence(s)

AK008712, AK011814, AK164169

Consensus CDS

CCDS84547.1

UniProtKB/TrEMBL

Q3UA17, Q9D050

Related

ENSMUSP00000118566.2, ENSMUST00000150232.8

Conserved Domains (1) summary

pfam00153
Location:137 → 201

Mito_carr; Mitochondrial carrier protein
NM_001317243.1 → NP_001304172.1 mitochondrial carrier homolog 2 isoform 3

Status: REVIEWED

Description

Transcript Variant: This variant (3) lacks an in-frame coding exon compared to variant 1. The resulting isoform (3) is shorter missing an internal 9 aa protein segment compared to isoform 1.

Source sequence(s)

AK008712, AK164169, CX243013

Consensus CDS

CCDS84548.1

UniProtKB/TrEMBL

A2AFW6, Q3UA17

Related

ENSMUSP00000107092.4, ENSMUST00000111467.4

Conserved Domains (1) summary

pfam00153
Location:119 → 183

Mito_carr; Mitochondrial carrier protein
NM_001317244.1 → NP_001304173.1 mitochondrial carrier homolog 2 isoform 4

Status: REVIEWED

Description

Transcript Variant: This variant (4) uses an alternate acceptor splice site at an internal exon, which results in translation initiation from an in-frame, downstream start codon compared to variant 1. The encoded isoform (4) has a shorter N-terminus compared to isoform 1.

Source sequence(s)

AK007757, AK008712, AK164169

UniProtKB/Swiss-Prot

Q791V5

Conserved Domains (1) summary

pfam00153
Location:1 → 65

Mito_carr; Mitochondrial carrier protein
NM_019758.3 → NP_062732.1 mitochondrial carrier homolog 2 isoform 1

See identical proteins and their annotated locations for NP_062732.1

Status: REVIEWED

Description

Transcript Variant: This variant (1) represents the predominant transcript and encodes two isoforms, which result from the use of alternative in-frame translation termination codons. The shorter isoform (1) results from translation termination at the upstream UGA stop codon, while the longer isoform (1x) results from UGA stop codon readthrough to the downstream UAG termination codon. This RefSeq represents the shorter isoform (1).

Source sequence(s)

AK008712, AK164169

Consensus CDS

CCDS16416.1

UniProtKB/Swiss-Prot

Q3TPS5, Q791V5, Q99LZ6, Q9D060, Q9D7Y2, Q9QZP3

UniProtKB/TrEMBL

Q3UA17

Related

ENSMUSP00000121851.2, ENSMUST00000136872.8

Conserved Domains (1) summary

pfam00153
Location:128 → 192

Mito_carr; Mitochondrial carrier protein

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

NC_000068.8 Reference GRCm39 C57BL/6J

Range

90677434..90697154

Download

GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_017319137.2 → XP_017174626.1 mitochondrial carrier homolog 2 isoform X1

Related

ENSMUST00000057216.15

Conserved Domains (1) summary

pfam00153
Location:1 → 65

Mito_carr; Mitochondrial carrier protein