Ubiquitin-like modifier-activating enzyme 1 (UBA1) is a multidomain enzyme and regulator of the ubiquitin proteasome system (UPS). It effectively marks proteins for degradation by the proteasome, acting as a gatekeeper of the Ub conjugation cascade by activating ubiquitin in a two-step process involving adenylation and thioester bond formation followed by transfer of ubiquitin to Cdc34 (an E2 ubiquitin-conjugating enzyme).
Mutations in the ubiquitin-like modifier activating enzyme 1 gene (UBA1), leading to reduced UBA1 expression, cause a rare form of Spinal Muscular Atrophy (SMA) known as X-linked SMA (XL-SMA).
Aminoacyl-tRNA synthetases, including GARS (Glycyl-tRNA synthetase), havebeen identified as downstream targets of UBA1. Dysregulation of UBA1/GARS pathways in spinal muscular atrophy results in a similar phenotype to GARS-dependent defects (such as Charcot-Marie-Tooth disease), perhaps indicating significant molecular and phenotypic overlap between spinal muscular atrophy and Charcot-Marie-Tooth disease.
Product Type:
NS Reagents Antibody
Antibody Type:
Polyclonal
Format:
100 µg in 100 µl PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
If you would like us to check if this antibody is likely to bind to this protein from a different species please contact us. We are happy to check for you.
Immunogen:
Partial length recombinant human UBA1 from the central region (less than 100 aa in length)
If you would like further information regarding the immunogen used in the production of this antibody or have a query about whether this antibody will bind to your protein/species please contact us and we can do the analysis for you.
Antibody Isotype:
IgG
Application Details:
WB 1:500-2000.
Antibody Number:
10123
Category:
Primary Antibodies
Other names:
A1S9, A1ST, GXP1, UBE1, A1S9T, AMCX1, POC20, SMAX2, UBA1A, UBE1X, CFAP124, Ubiquitin Like Modifier Activating Enzyme, Ubiquitin-Activating Enzyme E1, A1S9T And BN75 Temperature Sensitivity Complementing, Ubiquitin-Activating Enzyme E1 Homolog, Testicular Secretory Protein Li 63, POC20 Centriolar Protein Homolog, Protein A1S9.
Research Areas:
Neuroscience
NS Reagents Product Area:
Neuroscience
Molecular Weight:
118kDa (Intended as a general guide and does not allow for all isoforms and species variations)
Ubiquitin-like modifier-activating enzyme 1 (UBA1) is a multidomain enzyme and regulator of the ubiquitin proteasome system (UPS). It effectively marks proteins for degradation by the proteasome, acting as a gatekeeper of the Ub conjugation cascade by activating ubiquitin in a two-step process involving adenylation and thioester bond formation followed by transfer of ubiquitin to Cdc34 (an E2 ubiquitin-conjugating enzyme).
Mutations in the ubiquitin-like modifier activating enzyme 1 gene (UBA1), leading to reduced UBA1 expression, cause a rare form of Spinal Muscular Atrophy (SMA) known as X-linked SMA (XL-SMA).
Aminoacyl-tRNA synthetases, including GARS (Glycyl-tRNA synthetase), havebeen identified as downstream targets of UBA1. Dysregulation of UBA1/GARS pathways in spinal muscular atrophy results in a similar phenotype to GARS-dependent defects (such as Charcot-Marie-Tooth disease), perhaps indicating significant molecular and phenotypic overlap between spinal muscular atrophy and Charcot-Marie-Tooth disease.
Product Type:
NS Reagents Antibody
Antibody Type:
Polyclonal
Format:
50 µg in 50 µl PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
If you would like us to check if this antibody is likely to bind to this protein from a different species please contact us. We are happy to check for you.
Immunogen:
Partial length recombinant human UBA1 from the central region (less than 100 aa in length)
If you would like further information regarding the immunogen used in the production of this antibody or have a query about whether this antibody will bind to your protein/species please contact us and we can do the analysis for you.
Antibody Isotype:
IgG
Application Details:
WB 1:500-2000.
Antibody Number:
10123
Category:
Primary Antibodies
Other names:
A1S9, A1ST, GXP1, UBE1, A1S9T, AMCX1, POC20, SMAX2, UBA1A, UBE1X, CFAP124, Ubiquitin Like Modifier Activating Enzyme, Ubiquitin-Activating Enzyme E1, A1S9T And BN75 Temperature Sensitivity Complementing, Ubiquitin-Activating Enzyme E1 Homolog, Testicular Secretory Protein Li 63, POC20 Centriolar Protein Homolog, Protein A1S9.
Research Areas:
Neuroscience
NS Reagents Product Area:
Neuroscience
Molecular Weight:
118kDa (Intended as a general guide and does not allow for all isoforms and species variations)
Subcellular location:
Nucleus, Mitochondrion
Purification:
Affinity purification
Uniprot ID:
P22314
Gene ID:
7317
Gene symbol:
UBA1
Release Date:
Sep-19
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