Mouse Anti-TRNT1 Recombinant Antibody (CBYJT-4886) (CBMAB-T4432-YJ)

Name: Mouse Anti-TRNT1 Recombinant Antibody (CBYJT-4886)
SKU: CBMAB-T4432-YJ
Rating: 5 (1 reviews)

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Datasheet

SDS

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Basic Information

Host Animal

Mouse

Clone

CBYJT-4886

Application

WB, S-ELISA, ELISA

Immunogen

TRNT1 (AAH12537, 1 a.a. ~ 405 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa

Specificity

Human

Antibody Isotype

IgG2b, κ

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Buffer

PBS, pH 7.2

Storage

Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Epitope

aa 1-405

More Infomation

Target

Full Name

tRNA nucleotidyl transferase, CCA-adding, 1

Introduction

TRNT1 is a CCA-adding enzyme which belongs to the tRNA nucleotidyltransferase/poly(A) polymerase family. This necessary 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.

Entrez Gene ID

51095

UniProt ID

Q96Q11

Alternative Names

TRNA Nucleotidyl Transferase 1; TRNA Nucleotidyl Transferase, CCA-Adding, 1; ATP(CTP):TRNA Nucleotidyltransferase; TRNA CCA Nucleotidyl Transferase 1; Mt TRNA CCA-Pyrophosphorylase; Mt TRNA CCA-Diphosphorylase; Mt TRNA Adenylyltransferase; Mt CCA-Adding Enzyme; CCA-Adding Enzyme; Mitochondrial TRNA Nucleotidyl Transferase, CCA-Adding

Function

Isoform 1
Adds and repairs the conserved 3'-CCA sequence necessary for the attachment of amino acids to the 3' terminus of tRNA molecules, using CTP and ATP as substrates.
Isoform 2
Adds 2 C residues (CC-) to the 3' terminus of tRNA molecules instead of a complete CCA end as isoform 1 does (in vitro).

Biological Process

Biological Process mitochondrial tRNA 3'-end processing Source:UniProtKB1 Publication
Biological Process tRNA 3'-end processing Source:Reactome
Biological Process tRNA 3'-terminal CCA addition Source:UniProtKB1 Publication
Biological Process tRNA processing Source:GO_Central1 Publication

Cellular Location

Mitochondrion

Involvement in disease

Sideroblastic anemia with B-cell immunodeficiency, periodic fevers, and developmental delay (SIFD):
An autosomal recessive disease characterized by severe sideroblastic anemia with onset in the neonatal period or infancy, recurrent periodic fevers without an infectious etiology, B-cell lymphopenia and hypogammaglobulinemia. Affected individuals show delayed psychomotor development with variable neurodegeneration. Additional variable features include sensorineural hearing loss, retinitis pigmentosa, nephrocalcinosis, and cardiomyopathy.
Retinitis pigmentosa and erythrocytic microcytosis (RPEM):
An autosomal recessive disease characterized by retinitis pigmentosa, red blood cell microcytosis and anisocytosis with mild anemia.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Related Products

Mouse Anti-TRNT1 Recombinant Antibody (CBYJT-4885) (CAT#: CBMAB-T4431-YJ)

Isotype control

For research use only. Not intended for any clinical use.

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We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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