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Slc25a19 Gene Detail
Summary
  • Symbol
    Slc25a19
  • Name
    solute carrier family 25 (mitochondrial thiamine pyrophosphate carrier), member 19
  • Synonyms
    2900089E13Rik, DNC, MUP1, TPC
  • Feature Type
    protein coding gene
  • IDs
    MGI:1914533
    NCBI Gene: 67283
  • Alliance
  • Transcription Start Sites
    8 TSS
Location &
Maps
more
  • Sequence Map
    Chr11:115505004-115519121 bp, - strand
    From Ensembl annotation of GRCm39
  • View this region in JBrowse
  • Genome Browsers
  • Genetic Map
    Chromosome 11, 80.91 cM, cytoband E2
  • Mapping Data
    2 experiments
Strain
Comparison
more
  • SNPs within 2kb
    469 from dbSNP Build 142
  • Strain Annotations
    27
For selected strains:
Strain Gene Model ID Feature Type Coordinates Select Strains
C57BL/6J MGI_C57BL6J_1914533
protein coding gene Chr11:115504991-115519122 (-)
129S1/SvImJ ENSMUSGG00200054451
protein coding gene Chr11:112877738-112885094 (-)
129S1/SvImJ ENSMUSG00200049240
protein coding gene Chr11:112886468-112886637 (-)
A/J ENSMUSG00195029752
protein coding gene Chr11:112590396-112590565 (-)
A/J ENSMUSGG00195054947
protein coding gene Chr11:112581663-112589020 (-)
AKR/J ENSMUSG00220023766
protein coding gene Chr11:112689045-112689214 (-)
AKR/J ENSMUSGG00220054403
protein coding gene Chr11:112680302-112687669 (-)
BALB/cJ ENSMUSG00180051606
protein coding gene Chr11:112890821-112890990 (-)
BALB/cJ ENSMUSGG00180055089
protein coding gene Chr11:112882085-112889447 (-)
C3H/HeJ ENSMUSGG00175055091
protein coding gene Chr11:112833564-112840921 (-)
C3H/HeJ ENSMUSG00175033821
protein coding gene Chr11:112842297-112842466 (-)
C57BL/6NJ ENSMUSG00215044257
protein coding gene Chr11:112374921-112375090 (-)
C57BL/6NJ ENSMUSGG00215055263
protein coding gene Chr11:112366162-112373547 (-)
CAROLI/EiJ MGP_CAROLIEiJ_G0017413
protein coding gene Chr11:111455329-111469414 (-)
CAST/EiJ no annotation
CBA/J ENSMUSG00210048696
protein coding gene Chr11:112562285-112562454 (-)
CBA/J ENSMUSGG00210054988
protein coding gene Chr11:112553553-112560909 (-)
DBA/2J ENSMUSGG00185057406
protein coding gene Chr11:112873691-112881052 (-)
DBA/2J ENSMUSG00185021212
protein coding gene Chr11:112882426-112882595 (-)
FVB/NJ ENSMUSG00205028474
protein coding gene Chr11:112685029-112685198 (-)
FVB/NJ ENSMUSGG00205054099
protein coding gene Chr11:112676298-112683655 (-)
JF1/MsJ no annotation
LP/J ENSMUSG00230049592
protein coding gene Chr11:113935637-113935806 (-)
LP/J ENSMUSGG00230055304
protein coding gene Chr11:113926905-113934261 (-)
NOD/ShiLtJ ENSMUSGG00190054996
protein coding gene Chr11:112902225-112909583 (-)
NOD/ShiLtJ ENSMUSG00190045787
protein coding gene Chr11:112910957-112911126 (-)
NZO/HlLtJ ENSMUSGG00225055714
protein coding gene Chr11:115980381-115987737 (-)
NZO/HlLtJ ENSMUSG00225050406
protein coding gene Chr11:115989111-115989280 (-)
PWK/PhJ no annotation
SPRET/EiJ ENSMSPG00010041524
protein coding gene Chr11:113163357-113174867 (-)
WSB/EiJ no annotation



Homology
more
  • Human Ortholog
    SLC25A19, solute carrier family 25 member 19
  • Vertebrate Orthologs
    3
Vertebrate Orthology Source
Alliance of Genome Resources
  • Human Ortholog
    SLC25A19, solute carrier family 25 member 19
  • Synonyms
    DNC, MCPHA, MTPPT, MUP1, THMD3, THMD4, TPC
  • Links
    NCBI Gene ID: 60386
    UniProt: Q9HC21

  • Chr Location
    17q25.1; chr17:75272012-75289968 (-)  GRCh38

Human Diseases
more
  • Diseases
    2 with human SLC25A19 associations

Human Disease Mouse Models
      
IDs
IDs
Click on a disease name to see all genes associated with that disease.

Mutations,
Alleles, and
Phenotypes
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  • Phenotype Summary
    15 phenotypes from 1 allele in 1 genetic background
    2 images
    10 phenotype references
Phenotype Overview

adipose tissue
behavior/neurological
cardiovascular system
cellular
craniofacial
digestive/alimentary system
embryo
endocrine/exocrine glands
growth/size/body
hearing/vestibular/ear
hematopoietic system
homeostasis/metabolism
integument
immune system
limbs/digits/tail
liver/biliary system
mortality/aging
muscle
nervous system
pigmentation
renal/urinary system
reproductive system
respiratory system
skeleton
taste/olfaction
neoplasm
vision/eye

Click cells to view annotations.
Homozygous mutation of this gene results in lethality by E12, neural tube closure defects resulting in exencephaly and microcephaly, growth arrest, anemia, elevated alpha-ketoglutarate in amniotic fluid, and reduced thiamine pyrophosphate content in mitochondria.
Gene Ontology
(GO)
Classifications
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  • All GO Annotations
  • GO References
Molecular Function

carbohydrate derivative binding
cytoskeletal protein binding
DNA binding
enzyme regulator
hydrolase
ligase
lipid binding
oxidoreductase
RNA binding
signaling receptor activity
signaling receptor binding
transcription
transferase
transporter
Biological Process

carbohydrate derivative metabolism
cell differentiation
cell population proliferation
cellular component organization
DNA-templated transcription
establishment of localization
homeostatic process
immune system process
lipid metabolic process
programmed cell death
protein metabolic process
response to stimulus
signaling
system development
Cellular Component

cell projection
cytoplasmic vesicle
cytoskeleton
cytosol
endoplasmic reticulum
endosome
extracellular region
Golgi apparatus
mitochondrion
membraneless organelle
nucleus
organelle envelope
organelle lumen
plasma membrane
protein-containing complex
synapse
vacuole
Click cells to view annotations.
Expression
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Expression Overview

early conceptus
embryo ectoderm
embryo endoderm
embryo mesoderm
embryo mesenchyme
extraembryonic component
alimentary system
auditory system
branchial arches
cardiovascular system
connective tissue
endocrine system
exocrine system
hemolymphoid system
integumental system
limbs
liver and biliary system
musculoskeletal system
nervous system
olfactory system
reproductive system
respiratory system
urinary system
visual system
Click cells to view annotations.


  • Assay Results
  • Tissues
  • cDNA Data
  • Literature Summary
  • Comparison Matrix
  • Sequences &
    Gene Models
    less
    Representative SequencesLengthStrain/SpeciesFlank
    genomic ENSMUSG00000020744 Ensembl Gene Model | MGI Sequence Detail 14118 C57BL/6J ±  kb
    transcript ENSMUST00000178003 Ensembl | MGI Sequence Detail 2614 Not Applicable  
    polypeptide ENSMUSP00000137534 Ensembl | MGI Sequence Detail 318 Not Applicable  
    For the selected sequence
    Protein
    Information
    less
    • UniProt
      7 Sequences
    • Protein Ontology
      PR:000015014 mitochondrial thiamine pyrophosphate carrier
    • InterPro Domains
      IPR002067 Mitochondrial carrier protein
      IPR023395 Mitochondrial carrier protein domain superfamily
      IPR018108 Mitochondrial carrier protein, transmembrane region
    Molecular
    Reagents
    less
    • All nucleic 19
      cDNA 17
      Primer pair 2

      Microarray probesets 4
    References
    more
    • Summaries
      All 49
      Developmental Gene Expression 3
      Gene Ontology 11
      Phenotypes 10
    • Earliest
      J:335803 Bettendorff L, The compartmentation of phosphorylated thiamine derivatives in cultured neuroblastoma cells. Biochim Biophys Acta. 1994 May 26;1222(1):7-14
    • Latest
      J:360776 Di Michele M, et al., E4F1 coordinates pyruvate metabolism and the activity of the elongator complex to ensure translation fidelity during brain development. Nat Commun. 2025 Jan 2;16(1):67

    Contributing Projects:
    Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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    last database update
    09/01/2026
    MGI 6.24
    The Jackson Laboratory