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Mouse Anti-UBA1 Antibody (12BA8) (CBMAB-1716CQ)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
12BA8
Antibody Isotype
IgG2b
Application
ELISA, WB

Basic Information

Immunogen
Recombinant full length protein
Specificity
Human
Antibody Isotype
IgG2b
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!]

Format
Supernatant
Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
UBA1
Introduction
The protein encoded by this gene catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation. This gene complements an X-linked mouse temperature-sensitive defect in DNA synthesis, and thus may function in DNA repair. Diseases associated with UBA1 include Spinal Muscular Atrophy, X-Linked 2, Infantile and Spinal Muscular Atrophy. Among its related pathways are Proteolysis_Putative ubiquitin pathway and Protein ubiquitination.
Entrez Gene ID
7317
UniProt ID
P22314
Alternative Names
A1S9; A1ST; GXP1; UBE1; A1S9T; AMCX1; POC20; SMAX2; UBA1A; UBE1X; CFAP124
Function
Catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation through the ubiquitin-proteasome system (PubMed:1606621, PubMed:1447181, PubMed:33108101).
Activates ubiquitin by first adenylating its C-terminal glycine residue with ATP, and thereafter linking this residue to the side chain of a cysteine residue in E1, yielding a ubiquitin-E1 thioester and free AMP (PubMed:1447181).
Essential for the formation of radiation-induced foci, timely DNA repair and for response to replication stress. Promotes the recruitment of TP53BP1 and BRCA1 at DNA damage sites (PubMed:22456334).
Biological Process
Biological Process cellular response to DNA damage stimulus Source:UniProtKB1 Publication
Biological Process protein modification by small protein conjugation Source:GO_Central1 Publication
Biological Process protein ubiquitination Source:GO_Central1 Publication
Biological Process ubiquitin-dependent protein catabolic process Source:GO_Central1 Publication
Cellular Location
Cytoplasm
Mitochondrion
Nucleus
Isoform 1
Nucleus
Isoform 2
Cytoplasm
Involvement in disease
Spinal muscular atrophy X-linked 2 (SMAX2):
A lethal infantile form of spinal muscular atrophy, a neuromuscular disorder characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. Clinical features include hypotonia, areflexia, and multiple congenital contractures.
VEXAS syndrome (VEXAS):
A sporadic, often fatal, treatment-refractory inflammatory syndrome that develops in late adulthood. Clinical features include fevers, cytopenias, characteristic vacuoles in myeloid and erythroid precursor cells, dysplastic bone marrow, neutrophilic cutaneous and pulmonary inflammation, chondritis, and vasculitis. The disease affects only males and is associated with de novo somatic mutations.
PTM
ISGylated.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

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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