Human Mitochondrial thiamine pyrophosphate carrier (SLC25A19) ELISA kit; DNC; MCPHA; MUP1; TPC; mitochondrial uncoupling protein 1; solute carrier family 25 (mitochondrial deoxynucleotide carrier); member 19; solute carrier family 25; member 19; solute carrier family 25 (mitochondrial thiamine pyrophosphate carrier); member 19

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

Store all reagents at 2-8 degree C

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

Small volumes of SLC25A19 elisa kit 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.

MBS9328085 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the solute carrier family 25 (mitochondrial thiamine pyrophosphate carrier), member 19 (SLC25A19) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing SLC25A19. The ELISA analytical biochemical technique of the MBS9328085 kit is based on SLC25A19 antibody-SLC25A19 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect SLC25A19 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, SLC25A19. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

35,511 Da

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

mitochondrial thiamine pyrophosphate carrier; microcephaly, Amish; mitochondrial uncoupling protein 1; solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19

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

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

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.