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GeneBio Systems

Anti-SLC25A19 Antibody Picoband®

Anti-SLC25A19 Antibody Picoband®

SKU:A07902-1

Regular price $618.00 USD
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Size: 100 μg

Storage: Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.

Form: Lyophilized

Reactivity: Human

Applications: WB

Application Details: Western blot, 0.25-0.5μg/ml, Human

Gene Name: SLC25A19

Specificity:

Background: Mitochondrial thiamine pyrophosphate carrier is a protein that in humans is encoded by the SLC25A19 gene. 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.

Immunogen: A synthetic peptide corresponding to a sequence at the N-terminus of human SLC25A19, which shares 87.5% and 81.25% amino acid (aa) sequence identity with mouse and rat SLC25A19, respectively.

Clonality: Polyclonal

Contents: Each vial contains 4mg Trehalose, 0.9mg NaCl and 0.2mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Reference: 1. Dolce, V., Fiermonte, G., Runswick, M. J., Palmieri, F., Walker, J. E. The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals. Proc. Nat. Acad. Sci. 98: 2284-2288, 2001. 2. Iacobazzi, V., Ventura, M., Fiermonte, G., Prezioso, G., Rocchi, M., Palmieri, F. Genomic organization and mapping of the gene (SLC25A19) encoding the human mitochondrial deoxynucleotide carrier (DNC). Cytogenet. Cell Genet. 93: 40-42, 2001. 3. Lindhurst, M. J., Fiermonte, G., Song, S., Struys, E., De Leonardis, F., Schwarzberg, P. L., Chen, A., Castegna, A., Verhoeven, N., Mathews, C. K., Palmieri, F., Biesecker, L. G. Knockout of Slc25a19 causes mitochondrial thiamine pyrophosphate depletion, embryonic lethality, CNS malformations, and anemia. Proc. Nat. Acad. Sci. 103: 15927-15932, 2006.

Uniprot ID: Q9HC21

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 41 kDa

Calculated Molecular Weight: 49229 MW

Gene ID: 60386

Protein Name: Mitochondrial thiamine pyrophosphate carrier

Gene Full Name: solute carrier family 25 member 19

Synonyms: solute carrier family 25 member 19; DNC; MCPHA; MUP1; THMD3; THMD4; TPC; SLC25A19

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