Mitochondrial thiamine pyrophosphate carrier; Mitochondrial uncoupling protein 1; Solute carrier family 25 member 19; SLC25A19; DNC; MUP1

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This SLC25A19 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 236-263 amino acids from the C-terminal region of human SLC25A19.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-SLC25A19 antibody 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.

SLC25A19 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.

Metabolism; Signal Transduction

Western Blot (WB), ELISA (EIA)

WB~~1:1000

Western blot analysis of SLC25A19 Antibody (C-term) in mouse cerebellum,heart tissue lysates (35ug/lane).SLC25A19 (arrow) was detected using the purified Pab.

35511

solute carrier family 25 (mitochondrial thiamine pyrophosphate carrier), member 19

mitochondrial thiamine pyrophosphate carrier

Mitochondrial thiamine pyrophosphate carrier

DNC; MUP1??[Similar Products]

TPC_HUMAN

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]

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, multi-pass; Transporter; Mitochondrial; Membrane protein, integral; Transporter, SLC family

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

Spiegel, R., et al. Ann. Neurol. 66(3):419-424(2009)
Kang, J., et al. Mitochondrion 8(2):103-108(2008)
Lindhurst, M.J., et al. Proc. Natl. Acad. Sci. U.S.A. 103(43):15927-15932(2006)

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.