Product Name
SLC25A19, Blocking Peptide
Full Product Name
SLC25A19 Peptide - middle region
Product Gene Name
SLC25A19 blocking peptide
[Similar Products]
Product Synonym Gene Name
DNC; MCPHA; MUP1; TPC; THMD3; THMD4[Similar Products]
SLC25A19 peptide (MBS3232129) is used for blocking the activity of SLC25A19 antibody (MBS3207164)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence
VHRGSVYDAR EFSVHFVCGG LAACMATLTV HPVDVLRTRF AAQGEPKVYN
3D Structure
ModBase 3D Structure for Q9HC21
Form/Format
Lyophilized powder
Preparation and Storage
Add 100ul of sterile PBS. Final peptide concentration is 1 mg/ml in PBS. For longer periods of storage, store at -20 degree C. Avoid repeat freeze-thaw cycles.
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
This is a synthetic peptide designed for use in combination with anti-SLC25A19 Antibody, made
Target Description: 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.
Product Categories/Family for SLC25A19 blocking peptide
Peptide
Applications Tested/Suitable for SLC25A19 blocking peptide
Western Blot (WB)
NCBI/Uniprot data below describe general gene information for SLC25A19. It may not necessarily be applicable to this product.
NCBI Accession #
NP_068380
[Other Products]
NCBI GenBank Nucleotide #
NM_021734
[Other Products]
UniProt Primary Accession #
Q9HC21
[Other Products]
UniProt Related Accession #
Q9HC21[Other Products]
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: Transporter, SLC family; Membrane protein, multi-pass; Transporter; Mitochondrial; Membrane protein, integral
Chromosomal Location of Human Ortholog: 17q25.3
Cellular Component: mitochondrial inner membrane; integral to membrane; nucleus
Molecular Function: deoxynucleotide transmembrane transporter activity
Biological Process: deoxynucleotide transport; transmembrane transport
Disease: Thiamine Metabolism Dysfunction Syndrome 4 (bilateral Striatal Degeneration And Progressive Polyneuropathy Type); Microcephaly, Amish Type
Research Articles on SLC25A19
1. Pancreatic acinar cells Mitochondrial Thiamin Pyrophosphate uptake process is adaptively regulated by the prevailing thiamin level and this regulation is exerted at the level of transcription of the SLC25A19 gene and involves transcription factor binding affinity/epigenetic mechanisms.
Precautions
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Disclaimer
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