SLC25A36

Protein identityUniProt · HPA

UniProt accession

Q96CQ1

Gene

SLC25A36

Ensembl

ENSG00000114120

Chromosome

3

Canonical length

311 aa

Protein class

Predicted intracellular proteins, Predicted membrane proteins, Transporters

Subcellular location

Nucleoplasm,Mitochondria,Cytosol

OverviewNCBI Gene

Enables pyrimidine nucleotide transmembrane transporter activity. Involved in mitochondrial genome maintenance; pyrimidine nucleotide transport; and regulation of mitochondrial membrane potential. Located in mitochondrion. Implicated in familial hyperinsulinemic hypoglycemia 8. [provided by Alliance of Genome Resources, Jul 2025]

Canonical amino-acid sequenceUniProt

311 residues, UniProt reviewed canonical sequence.

>Q96CQ1|SLC25A36
     1  MSQRDTLVHL FAGGCGGTVG AILTCPLEVV KTRLQSSSVT LYISEVQLNT MAGASVNRVV
    61  SPGPLHCLKV ILEKEGPRSL FRGLGPNLVG VAPSRAIYFA AYSNCKEKLN DVFDPDSTQV
   121  HMISAAMAGF TAITATNPIW LIKTRLQLDA RNRGERRMGA FECVRKVYQT DGLKGFYRGM
   181  SASYAGISET VIHFVIYESI KQKLLEYKTA STMENDEESV KEASDFVGMM LAAATSKTCA
   241  TTIAYPHEVV RTRLREEGTK YRSFFQTLSL LVQEEGYGSL YRGLTTHLVR QIPNTAIMMA
   301  TYELVVYLLN G

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against SLC25A36 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability

Other membrane

Secreted

No

Transmembrane segments

6

Mean surface accessibility (rSASA)

0.32

Highest tissue expression

58 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about SLC25A36.

Disease | AllUniProt

Conditions SLC25A36 is implicated in, by any mechanism.

• Hyperinsulinemic hypoglycemia, familial, 8 (HHF8) MIM:620211

Disease | GeneticClinVar

2 pathogenic / likely-pathogenic of 44 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Hyperinsulinemic hypoglycemia, familial, 8

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)

0.72

gnomAD pLI

0.03

gnomAD missense Z

1.92

DepMap mean gene effect

-0.06

DepMap dependency class

selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 9% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

• pyrimidine nucleotide import into mitochondrion

Molecular functions

• pyrimidine nucleotide transmembrane transporter activity

Cellular components

• mitochondrial inner membrane
• mitochondrion

Protein domainsUniProt · Pfam · InterPro

• Mitochondrial carrier protein
• Mitochondrial carrier protein, transmembrane region
• Mitochondrial carrier protein domain superfamily
• Solute carrier family 25 member 33/36-like
• Mitochondrial carrier protein

KeywordsUniProt

• Membrane
• Mitochondrion
• Mitochondrion inner membrane
• Repeat
• Transmembrane
• Transmembrane helix
• Transport