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Anti-TRNT1 Antibody Picoband®, PE Conjugate

Boster Bio Anti-TRNT1 Antibody Picoband® catalog # A06030-1. Tested in ELISA, IHC, WB applications. This antibody reacts with Human. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Product Specifications

Background

tRNA-nucleotidyltransferase 1, is an enzyme that in humans is encoded by the TRNT1 gene. The protein encoded by this gene is a CCA-adding enzyme which belongs to the tRNA nucleotidyltransferase/poly(A) polymerase family. This essential enzyme functions by catalyzing the addition of the conserved nucleotide triplet CCA to the 3' terminus of tRNA molecules. Mutations in this gene result in sideroblastic anemia with B-cell immunodeficiency, periodic fevers, and developmental delay. Alternative splicing results in multiple transcript variants.

Synonyms

Tricarboxylate transport protein, mitochondrial; Citrate transport protein; CTP; Solute carrier family 25 member 1; Tricarboxylate carrier protein; SLC25A1; SLC20A3

Gene Name

solute carrier family 25 member 1

Gene ID

51095

UniProt

Q96Q11

Host

Rabbit

Reactivity

Human

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

E.coli-derived human TRNT1 recombinant protein (Position: M1-Q410).

Clonality

Polyclonal

Tissue Specificity

Detected in brain. Detected at very much lower levels in heart, lung, placenta and skeletal muscle. Highly expressed in cerebellum, but also found in frontal cortex, hippocampus and basal ganglia.

Applications

WB,IHC,ELISA

Purification

Immunogen affinity purified.

Concentration

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

Form

Lyophilized

Reconstitution

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

Function

Citrate transporter that mediates the exchange of mitochondrial citrate for cytosolic malate. Also able to mediate the exchange of citrate for isocitrate, phosphoenolpyruvate, cis- but not trans-aconitate and to a lesser extend maleate and succinate. Important for the bioenergetics of hepatic cells as it provides a carbon source for fatty acid and sterol biosyntheses, and NAD+ for the glycolytic pathway. Required for proper neuromuscular junction formation (Probable).

Components

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

References & Citations

1. Chakraborty, P. K., Schmitz-Abe, K., Kennedy, E. K., Mamady, H., Naas, T., Durie, D., Campagna, D. R., Lau, A., Sendamarai, A. K., Wiseman, D. H., May, A., Jolles, S., and 23 others. Mutations in TRNT1 cause congenital sideroblastic anemia with immunodeficiency, fevers, and developmental delay (SIFD). Blood 124: 2867-2871, 2014. 2. DeLuca, A. P., Whitmore, S. S., Barnes, J., Sharma, T. P., Westfall, T. A., Scott, C. A., Weed, M. C., Wiley, J. S., Wiley, L. A., Johnston, R. M., Schnieders, M. J., Lentz, S. R., Tucker, B. A., Mullins, R. F., Scheetz, T. E., Stone, E. M., Slusarski, D. C. Hypomorphic mutations in TRNT1 cause retinitis pigmentosa with erythrocytic microcytosis. Hum. Molec. Genet. 25: 44-56, 2016. 3. Lizano, E., Schuster, J., Muller, M., Kelso, J., Morl, M. A splice variant of the human CCA-adding enzyme with modified activity. J. Molec. Biol. 366: 1258-1265, 2007.

Storage Conditions

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 freezing and thawing.

Calculated Molecular Weight

80420 MW

Observed Molecular Weight

50 kDa

Fragment

Rabbit IgG

Applications Notes

Western blot, 0.25-0.5 μg/ml, Human<br> Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml, Human<br> ELISA, 0.1-0.5 μg/ml, -<br>

Subcellular Location

Mitochondrion inner membrane. Multi-pass membrane protein.

Protein Name

Long-chain-fatty-acid--CoA ligase 3

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