UBE1 / UBA1 recombinant protein

Recombinant Human UBE1 / UBA1 Protein (His & GST tag)

Cat. Num. AAA2545609

• Gene Names: UBA1; A1S9; A1ST; GXP1; UBE1; A1S9T; AMCX1; POC20; SMAX2; UBA1A; UBE1X; CFAP124
• Purity: > 96 % as determined by SDS-PAGE
• Synonyms: UBE1 / UBA1; Recombinant Human UBE1 / UBA1 Protein (His & GST tag); CTD-2522E6.1; A1S9; A1S9T; A1ST; AMCX1; GXP1; POC20; SMAX2; UBA1A; UBE1; UBE1X; UBE1 / UBA1 recombinant protein

• Host: Baculovirus-Insect Cells
• Purity/Purification: > 96 % as determined by SDS-PAGE
• Form/Format: Lyophilized from sterile 50mM Tris, 100mM NaCl, pH 7.4, 10% gly, 0.5mM GSH
• Sequence Length: 1058

• Application Notes: The recombinant human UBA1/GST chimera consists of 1294 amino acids and has a calculated molecular mass of 146 KDa. It migrates as an approximately 130 KDa band in SDS-PAGE under reducing conditions.
• Predicted N Terminal: Met
• Endotoxin: < 1.0 EU per mug of the protein as determined by the LAL method
• Preparation and Storage: Samples are stable for up to twelve months from date of receipt at -70 degree C

SDS-PAGE

Related Product Information for UBE1 / UBA1 recombinant protein

Background: UBE1, also known as UBA1, belongs to the ubiquitin-activating E1 family. UBE1 gene complements an X-linked mouse temperature-sensitive defect in DNA synthesis, and thus may function in DNA repair. It is part of a gene cluster on chromosome Xp11.23. UBE1 catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation. It also catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation 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 an ubiquitin-E1 thioester and free AMP. Defects in UBA1 can cause spinal muscular atrophy X-linked type 2 (SMAX2), also known as X-linked lethal infantile spinal muscular atrophy, distal X-linked arthrogryposis multiplex congenita or X-linked arthrogryposis type 1 (AMCX1). Spinal muscular atrophy refers to a group of neuromuscular disorders characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. SMAX2 is a lethal infantile form presenting with hypotonia, areflexia, and multiple congenital contractures.

Description: A DNA sequence encoding the human UBA1 (NP_003325.2) (Ser 2-Arg 1058) was fused with the N-terminal polyhistidine-tagged GST tag at the N-terminus.

NCBI and Uniprot Product Information

• NCBI GI #: 23510338
• NCBI GeneID: 7317
• NCBI Accession #: NP_003325.2
• NCBI GenBank Nucleotide #: NM_003334.3
• UniProt Accession #: P22314; Q5JRR8; Q96E13
• Molecular Weight: 113,800 Da
• NCBI Official Full Name: ubiquitin-like modifier-activating enzyme 1
• NCBI Official Synonym Full Names: ubiquitin like modifier activating enzyme 1
• NCBI Official Symbol: UBA1
• NCBI Official Synonym Symbols: A1S9; A1ST; GXP1; UBE1; A1S9T; AMCX1; POC20; SMAX2; UBA1A; UBE1X; CFAP124
• NCBI Protein Information: ubiquitin-like modifier-activating enzyme 1
• UniProt Protein Name: Ubiquitin-like modifier-activating enzyme 1
• UniProt Gene Name: UBA1
• UniProt Synonym Gene Names: A1S9T; UBE1

NCBI Description

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. It is part of a gene cluster on chromosome Xp11.23. Alternatively spliced transcript variants that encode the same protein have been described. [provided by RefSeq, Jul 2008]

Uniprot Description

UBE1: an enzyme of the ubiquitin-activating E1 family. Adenylates ubiquitin at its carboxy-terminal glycine residue and thereafter links this residue to the side chain of a cysteine residue in E1, yielding an ubiquitin-E1 thiolester. There are two active sites within the E1 molecule, allowing it to accomodate two ubiquitin moieties at a time, with a new ubiquitin forming an adenylate intermediate as the previous one is transferred to the thiol site. Complements an X-linked mouse temperature-sensitive defect in DNA synthesis, and thus may function in DNA repair.

Protein type: EC 6.3.2.19; Ubiquitin conjugating system; Ubiquitin ligase

Chromosomal Location of Human Ortholog: Xp11.3

Cellular Component: cytoplasm; cytosol; desmosome; endosome membrane; heterochromatin; lysosomal membrane; mitochondrion; nucleus; rough endoplasmic reticulum membrane

Molecular Function: protein binding; ubiquitin activating enzyme activity; ubiquitin-protein ligase activity

Biological Process: modification-dependent protein catabolic process; response to DNA damage stimulus

Disease: Spinal Muscular Atrophy, X-linked 2