Product Name
SLC25A19, Blocking Peptide
Full Product Name
SLC25A19 Antibody (C-term) Blocking Peptide
Product Synonym Names
Mitochondrial thiamine pyrophosphate carrier; Mitochondrial uncoupling protein 1; Solute carrier family 25 member 19; SLC25A19; DNC; MUP1
Product Gene Name
SLC25A19 blocking peptide
[Similar Products]
Product Synonym Gene Name
DNC; MUP1[Similar Products]
Antibody/Peptide Pairs
SLC25A19 peptide (MBS9225595) is used for blocking the activity of SLC25A19 antibody (MBS9214793)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q9HC21
Form/Format
Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
Cellular Location
Mitochondrion inner membrane; Multi-pass membrane protein
Tissue Location
Expressed in all tissues examined except for placenta. Highest levels in colon, kidney, lung, testis, spleen, and brain.
Preparation and Storage
Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.
Other Notes
Small volumes of SLC25A19 blocking peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
SLC25A19 blocking peptide
Mitochondrial transporter mediating uptake of thiamine pyrophosphate (ThPP) into mitochondria.
NCBI/Uniprot data below describe general gene information for SLC25A19. It may not necessarily be applicable to this product.
NCBI Accession #
Q9HC21.1
[Other Products]
UniProt Primary Accession #
Q9HC21
[Other Products]
UniProt Secondary Accession #
Q6V9R7; E9PF74[Other Products]
UniProt Related Accession #
Q9HC21[Other Products]
Molecular Weight
29,217 Da
NCBI Official Full Name
Mitochondrial thiamine pyrophosphate carrier
NCBI Official Synonym Full Names
solute carrier family 25 member 19
NCBI Official Symbol
SLC25A19 [Similar Products]
NCBI Official Synonym Symbols
DNC; TPC; MUP1; MCPHA; THMD3; THMD4
[Similar Products]
NCBI Protein Information
mitochondrial thiamine pyrophosphate carrier
UniProt Protein Name
Mitochondrial thiamine pyrophosphate carrier
UniProt Synonym Protein Names
Mitochondrial uncoupling protein 1; Solute carrier family 25 member 19
Protein Family
Mitochondrial thiamine pyrophosphate carrier
UniProt Gene Name
SLC25A19 [Similar Products]
UniProt Synonym Gene Names
DNC; MUP1 [Similar Products]
UniProt Entry Name
TPC_HUMAN
NCBI Summary for SLC25A19
This gene encodes a mitochondrial protein that is a member of the solute carrier family. Although this protein was initially thought to be the mitochondrial deoxynucleotide carrier involved in the uptake of deoxynucleotides into the matrix of the mitochondria, further studies have demonstrated that this protein instead functions as the mitochondrial thiamine pyrophosphate carrier, which transports thiamine pyrophosphates into mitochondria. Mutations in this gene cause microcephaly, Amish type, a metabolic disease that results in severe congenital microcephaly, severe 2-ketoglutaric aciduria, and death within the first year. Multiple alternatively spliced variants, encoding the same protein, have been identified for this gene. [provided by RefSeq, Jul 2008]
UniProt Comments for SLC25A19
SLC25A19: Mitochondrial transporter mediating uptake of thiamine pyrophosphate (ThPP) into mitochondria. Defects in SLC25A19 are the cause of microcephaly Amish type (MCPHA); also known as Amish lethal microcephaly. MCPHA is an autosomal recessive metabolic disorder characterized by severe congenital microcephaly, severe 2- ketoglutaric aciduria and death within the first year. Defects in SLC25A19 are the cause of striatal necrosis bilateral and progressive polyneuropathy (SNBPP). A disease characterized by recurrent episodes of flaccid paralysis and encephalopathy associated with bilateral striatal necrosis and chronic progressive polyneuropathy. Belongs to the mitochondrial carrier family.
Protein type: Membrane protein, integral; Transporter, SLC family; Transporter; Membrane protein, multi-pass; Mitochondrial
Chromosomal Location of Human Ortholog: 17q25.3
Cellular Component: nucleus
Molecular Function: deoxynucleotide transmembrane transporter activity; structural constituent of ribosome
Biological Process: positive regulation of defense response to virus by host; translation
Disease: Microcephaly, Amish Type; Thiamine Metabolism Dysfunction Syndrome 4 (bilateral Striatal Degeneration And Progressive Polyneuropathy Type)
Research Articles on SLC25A19
1. Chronic alcohol exposure negatively impacts pancreatic mitochondrial thiamin pyrophosphate transport, and this effect is exerted, at least in part, at the level of Slc25a19 transcription and appears to involve an epigenetic mechanism.
Precautions
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Disclaimer
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