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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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SLC25A16
solute carrier family 25 member 16
Chromosome 10 · 10q21.3
NCBI Gene: 8034Ensembl: ENSG00000122912.16HGNC: HGNC:10986UniProt: B4DHV9
26PubMed Papers
20Diseases
0Drugs
1Pathogenic Variants
FUNCTIONAL ROLE
Transporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
mitochondrial inner membranecoenzyme A transmembrane transporter activitytransmembrane transporter activitymitochondrial coenzyme A transmembrane transportinherited isolated nail anomalyIsolated nail anomalydrug dependenceHydrocephalus
✦AI Summary

SLC25A16 is a mitochondrial inner membrane transporter that regulates coenzyme A (CoA) homeostasis between the cytosol and mitochondria 1. The protein mediates import of free CoA from the cytosol into the mitochondrial matrix 2, supporting an enriched mitochondrial CoA pool essential for energy metabolism pathways including the TCA cycle and fatty acid oxidation 2. Alternatively, SLC25A16 may function in exporting 3'-dephospho-CoA from mitochondria to the cytosol 3. This CoA compartmentalization enables spatial regulation of anabolic and catabolic processes 2. Diseases involving SLC25A16 dysfunction have emerged from multiple genetic studies. A genome-wide association study identified SLC25A16 variants associated with stimulant dependence in African-ancestry populations (rs2394476, p = 3.42 × 10⁻¹⁰) 4. SLC25A16 was identified as a core druggable target in diabetic nephropathy, a mitochondrial dysfunction-related kidney disease 5. Additionally, SLC25A16 variants were identified as candidate genes in cerebral visual impairment cases 6, and altered SLC25A16 expression was associated with birth weight changes following prenatal metabolic disruption 7. These findings suggest SLC25A16's role in metabolic diseases warrants further clinical investigation.

Sources cited
1
SLC25A16 is a mitochondrial inner membrane transporter regulating CoA homeostasis and mediates CoA import
PMID: 11158296
2
SLC25A16 is critical for mitochondrial import of free CoA supporting TCA cycle and fatty acid oxidation
PMID: 40925986
3
SLC25A16 may function in export of 3'-dephospho-CoA from mitochondrial matrix to cytosol
PMID: 36316571
4
SLC25A16 variant (rs2394476) is genome-wide significant for stimulant dependence in African-ancestry populations
PMID: 34226506
5
SLC25A16 identified as core druggable target for diabetic nephropathy via Mendelian randomization analysis
PMID: 40125968
6
SLC25A16 variants identified as candidate genes in cerebral visual impairment cases
PMID: 26350515
7
SLC25A16 expression altered in association with birth weight following prenatal metabolic disruption
PMID: 37108768
Disease Associationsⓘ20
inherited isolated nail anomalyOpen Targets
0.33Weak
Isolated nail anomalyOpen Targets
0.33Weak
drug dependenceOpen Targets
0.28Weak
HydrocephalusOpen Targets
0.27Weak
secondary malignant neoplasmOpen Targets
0.27Weak
Rare genetic intellectual disability with developmental anomalyOpen Targets
0.27Weak
diabetic eye diseaseOpen Targets
0.05Suggestive
breast cancerOpen Targets
0.05Suggestive
neoplasmOpen Targets
0.03Suggestive
type 2 diabetes mellitusOpen Targets
0.03Suggestive
Abnormality of the skeletal systemOpen Targets
0.03Suggestive
Hutchinson-Gilford progeria syndromeOpen Targets
0.02Suggestive
acute myeloid leukemiaOpen Targets
0.02Suggestive
in situ carcinomaOpen Targets
0.01Suggestive
progeroid syndromeOpen Targets
0.01Suggestive
triple-negative breast cancerOpen Targets
0.01Suggestive
Alzheimer diseaseOpen Targets
0.01Suggestive
candidiasisOpen Targets
0.01Suggestive
chronic kidney diseaseOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
Pathogenic Variants1
NM_152707.4(SLC25A16):c.793C>T (p.Arg265Cys)Likely pathogenic
Cerebral visual impairment and intellectual disability
★☆☆☆2015→ Residue 265
View on ClinVar ↗
Related Genes
RPL18Protein interaction100%TRIM13Protein interaction79%MTCH2Protein interaction79%MTCH1Protein interaction78%SLC25A42Shared pathway67%SLC25A43Shared pathway50%
Tissue Expression6 tissues
Liver
100%
Lung
39%
Bone Marrow
36%
Heart
31%
Brain
28%
Ovary
22%
Gene Interaction Network
Click a node to explore
SLC25A16RPL18TRIM13MTCH2MTCH1SLC25A42SLC25A43
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt P16260
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.30LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.79 [0.49–1.30]
RankingsWhere SLC25A16 stands among ~20K protein-coding genes
  • #12,876of 20,598
    Most Researched26
  • #5,305of 5,498
    Most Pathogenic Variants1
  • #13,715of 17,882
    Most Constrained (LOEUF)1.30
Genes detectedSLC25A16
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Combining mitochondrial proteomes and Mendelian randomization to identify novel therapeutic targets for diabetic nephropathy.
PMID: 40125968
Ren Fail · 2025
1.00
2
Cellular pan-chain acyl-CoA profiling reveals SLC25A42/SLC25A16 in mitochondrial CoA import and metabolism.
PMID: 40925986
Nat Metab · 2025
0.90
3
Genome-wide association study of stimulant dependence.
PMID: 34226506
Transl Psychiatry · 2021
0.80
4
Novel genetic causes for cerebral visual impairment.
PMID: 26350515
Eur J Hum Genet · 2016
0.70
5
Prenatal Exposure to Metabolism-Disrupting Chemicals, Cord Blood Transcriptome Perturbations, and Birth Weight in a Belgian Birth Cohort.
PMID: 37108768
Int J Mol Sci · 2023
0.60