Anti-TRNT1 Antibody Picoband®

SKU: A06030-1

Size: 100 μg

Storage: 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.

Form: Lyophilized

Reactivity: Human

Applications: WB,IHC,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human
Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: TRNT1

Specificity:

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.

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

Clonality: Polyclonal

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

Purification: Immunogen affinity purified.

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

Reference: 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.

Uniprot ID: Q96Q11

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: 50 kDa

Calculated Molecular Weight: 80420 MW

Gene ID: 51095

Protein Name: CCA tRNA nucleotidyltransferase 1, mitochondrial

Gene Full Name: tRNA nucleotidyl transferase 1

Synonyms: TRNT1; CGI-47; CCA tRNA nucleotidyltransferase 1, mitochondrial; Mitochondrial tRNA nucleotidyl transferase, CCA-adding; mt CCA-adding enzyme; mt tRNA CCA-diphosphorylase; mt tRNA CCA-pyrophosphorylase; mt tRNA adenylyltransferase