Mitochondrial 2-oxoglutarate/malate carrier protein

Last updated
SLC25A11
Identifiers
Aliases SLC25A11 , OGC, SLC20A4, solute carrier family 25 member 11, PGL6
External IDs OMIM: 604165 MGI: 1915113 HomoloGene: 2637 GeneCards: SLC25A11
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_003562
NM_001165417
NM_001165418

NM_024211

RefSeq (protein)

NP_001158889
NP_001158890
NP_003553

NP_077173

Location (UCSC) Chr 17: 4.94 – 4.94 Mb Chr 11: 70.54 – 70.54 Mb
PubMed search [3] [4]
Wikidata
View/Edit Human View/Edit Mouse

Mitochondrial 2-oxoglutarate/malate carrier protein is a protein that in humans is encoded by the SLC25A11 gene. [5] [6] [7] Inactivating mutations in this gene predispose to metastasic paraganglioma. [8]

Contents

See also

Related Research Articles

<span class="mw-page-title-main">Mitochondrial carrier</span>

Mitochondrial carriers are proteins from solute carrier family 25 which transfer molecules across the membranes of the mitochondria. Mitochondrial carriers are also classified in the Transporter Classification Database. The Mitochondrial Carrier (MC) Superfamily has been expanded to include both the original Mitochondrial Carrier (MC) family and the Mitochondrial Inner/Outer Membrane Fusion (MMF) family.

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<span class="mw-page-title-main">Phosphate carrier protein, mitochondrial</span>

Phosphate carrier protein, mitochondrial is a protein that in humans is encoded by the SLC25A3 gene. The encoded protein is a transmembrane protein located in the mitochondrial inner membrane and catalyzes the transport of phosphate ions across it for the purpose of oxidative phosphorylation. There are two significant isoforms of this gene expressed in human cells, which differ slightly in structure and function. Mutations in this gene can cause mitochondrial phosphate carrier deficiency (MPCD), a fatal disorder of oxidative phosphorylation symptomized by lactic acidosis, neonatal hypotonia, hypertrophic cardiomyopathy, and death within the first year of life.

<span class="mw-page-title-main">SLC26A6</span> Protein-coding gene in the species Homo sapiens

Solute carrier family 26 member 6 is a protein that in humans is encoded by the SLC26A6 gene. It is an anion-exchanger expressed in the apical membrane of the kidney proximal tubule, the apical membranes of the duct cells in the pancreas, and the villi of the duodenum.

<span class="mw-page-title-main">Y+L amino acid transporter 1</span> Protein-coding gene in the species Homo sapiens

Y+L amino acid transporter 1 is a protein that in humans is encoded by the SLC7A7 gene.

<span class="mw-page-title-main">Peroxisomal membrane protein PMP34</span> Protein found in humans

Peroxisomal membrane protein PMP34 is a protein that in humans is encoded by the SLC25A17 gene.

<span class="mw-page-title-main">UDP-galactose translocator</span> Protein found in humans

UDP-galactose translocator is a protein that in humans is encoded by the SLC35A2 gene.

<span class="mw-page-title-main">Mitochondrial uncoupling protein 4</span> Protein-coding gene in the species Homo sapiens

Mitochondrial uncoupling protein 4 is a protein that in humans is encoded by the SLC25A27 gene.

<span class="mw-page-title-main">Mitochondrial thiamine pyrophosphate carrier</span> Protein-coding gene in the species Homo sapiens

Mitochondrial thiamine pyrophosphate carrier is a protein that in humans is encoded by the SLC25A19 gene.

<span class="mw-page-title-main">Mitochondrial dicarboxylate carrier</span> Mammalian protein found in Homo sapiens

The mitochondrial dicarboxylate carrier (DIC) is an integral membrane protein encoded by the SLC25A10 gene in humans that catalyzes the transport of dicarboxylates such as malonate, malate, and succinate across the inner mitochondrial membrane in exchange for phosphate, sulfate, and thiosulfate by a simultaneous antiport mechanism, thus supplying substrates for the Krebs cycle, gluconeogenesis, urea synthesis, fatty acid synthesis, and sulfur metabolism.

<span class="mw-page-title-main">Calcium-binding mitochondrial carrier protein Aralar1</span> Protein-coding gene in the species Homo sapiens

Calcium-binding mitochondrial carrier protein Aralar1 is a protein that in humans is encoded by the SLC25A12 gene. Aralar is an integral membrane protein located in the inner mitochondrial membrane. Its primary function as an antiporter is the transport of cytoplasmic glutamate with mitochondrial aspartate across the inner mitochondrial membrane, dependent on the binding of one calcium ion. Mutations in this gene cause early infantile epileptic encephalopathy 39 (EIEE39), symptomized by global hypomyelination of the central nervous system, refractory seizures, and neurodevelopmental impairment. This gene has connections to autism.

<span class="mw-page-title-main">Vesicular inhibitory amino acid transporter</span> Protein-coding gene in the species Homo sapiens

Vesicular inhibitory amino acid transporter is a protein that in humans is encoded by the SLC32A1 gene.

<span class="mw-page-title-main">SLC22A7</span> Protein-coding gene in the species Homo sapiens

Solute carrier family 22 member 7 is a protein that in humans is encoded by the gene SLC22A7.

<span class="mw-page-title-main">SLC25A39</span> Protein-coding gene in the species Homo sapiens

Solute carrier family 25 member 39 is a protein that in humans is encoded by the SLC25A39 gene. The protein has been shown to be necessary for the import of the major antioxidant glutathione into the mitochondria.

<span class="mw-page-title-main">Tricarboxylate transport protein, mitochondrial</span> Mammalian protein found in Homo sapiens

Tricarboxylate transport protein, mitochondrial, also known as tricarboxylate carrier protein and citrate transport protein (CTP), is a protein that in humans is encoded by the SLC25A1 gene. SLC25A1 belongs to the mitochondrial carrier gene family SLC25. High levels of the tricarboxylate transport protein are found in the liver, pancreas and kidney. Lower or no levels are present in the brain, heart, skeletal muscle, placenta and lung.

<span class="mw-page-title-main">ADP/ATP translocase 3</span> Protein-coding gene in humans

ADP/ATP translocase 3, also known as solute carrier family 25 member 6, is a protein that in humans is encoded by the SLC25A6 gene.

<span class="mw-page-title-main">SLC6A18</span> Protein-coding gene in the species Homo sapiens

Solute carrier family 6, member 18 also known as SLC6A18 is a protein which in humans is encoded by the SLC6A18 gene.

<span class="mw-page-title-main">Calcium-binding mitochondrial carrier protein SCaMC-1</span> Protein-coding gene in the species Homo sapiens

Calcium-binding mitochondrial carrier protein SCaMC-1 is a protein that in humans is encoded by the SLC25A24 gene.

<span class="mw-page-title-main">SLC25A22</span> Protein-coding gene in the species Homo sapiens

Solute carrier family 25 member 22 is a protein that in humans is encoded by the SLC25A22 gene. This gene encodes a mitochondrial glutamate carrier. Mutations in this gene are associated with early infantile epileptic encephalopathy. Expression of this gene is increased in colorectal tumor cells.

<span class="mw-page-title-main">SLC25A46</span> Protein-coding gene in the species Homo sapiens

Solute carrier family 25 member 46 is a protein that in humans is encoded by the SLC25A46 gene. This protein is a member of the SLC25 mitochondrial solute carrier family. It is a transmembrane protein located in the mitochondrial outer membrane involved in lipid transfer from the endoplasmic reticulum (ER) to mitochondria. Mutations in this gene result in neuropathy and optic atrophy.

References

  1. 1 2 3 GRCh38: Ensembl release 89: ENSG00000108528 - Ensembl, May 2017
  2. 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000014606 - Ensembl, May 2017
  3. "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. Piccininni S, Iacobazzi V, Lauria G, Rocchi M, Palmieri F (Mar 1999). "Assignment of the oxoglutarate carrier gene (SLC20A4) to human chromosome 17p13.3". Cytogenet Cell Genet. 83 (3–4): 256–7. doi:10.1159/000015198. PMID   10072597. S2CID   36221506.
  6. Iacobazzi V, Palmieri F, Runswick MJ, Walker JE (Jan 1993). "Sequences of the human and bovine genes for the mitochondrial 2-oxoglutarate carrier". DNA Seq. 3 (2): 79–88. doi:10.3109/10425179209034000. PMID   1457818.
  7. "Entrez Gene: SLC25A11 solute carrier family 25 (mitochondrial carrier; oxoglutarate carrier), member 11".
  8. Buffet, Alexandre; Morin, Aurelie; Castro-Vega, Luis-Jaime; Habarou, Florence; Lussey-Lepoutre, Charlotte; Letouzé, Eric; Lefebvre, Hervé; Guilhem, Isabelle; Magalie, Haissaguerre (2018-02-05). "Germline mutations in the mitochondrial 2-oxoglutarate/malate carrier SLC25A11 gene confer a predisposition to metastatic paragangliomas". Cancer Research. 78 (8): 1914–1922. doi: 10.1158/0008-5472.CAN-17-2463 . ISSN   1538-7445. PMID   29431636.

Further reading