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貨期:
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用途:
For Research Use Only. Not for use in diagnostic or therapeutic procedures.
Ubiquitin-like modifier which can be covalently attached via an isopeptide bond to lysine residues of substrate proteins as a monomer or a lysine-linked polymer. The so-called ufmylation, requires the UFM1-activating E1 enzyme UBA5, the UFM1-conjugating E2 enzyme UFC1, and the UFM1-ligase E3 enzyme UFL1. Ufmylation is involved in reticulophagy (also called ER-phagy) induced in response to endoplasmic reticulum stress. Ufmylation of TRIP4 regulates nuclear receptors-mediated transcription.
基因功能參考文獻:
UFM1 His 70 resembles UBA5 His336 and enters a negatively charged pocked on the other UFM1 molecule. PMID: 28360427
Ufmylation may participate in regulation of the vascular smooth muscle cells phenotypic switch and endothelial cell injury, which may help in the understanding of vascular remodeling. PMID: 29461087
These findings explicitly elucidate the role of UBA5 dimerization in UFM1 activation. PMID: 27653677
This study, focused on identifying the mutated gene in patients with hypomyelination with atrophy of the basal ganglia and cerebellum without TUBB4A mutations, revealed a homozygous 3-bp deletion in the UFM1 promoter area, which perfectly segregates with the disease. PMID: 28931644
Meta analysis identified a new gene, UFM1, that is associated with low von Willebrand factor levels. PMID: 26486471
in the absence of UFSP2, a deconjugating enzyme for UFM1, ectopic expression of both UFL1 and UFBP1, which serve as the E3-ligase complex for the UFM1-system, dramatically increases UFM1-conjugate formation at the endoplasmic reticulum. PMID: 27926783
data imply that the combination of a hypomorphic p.Ala371Thr variant in trans with a loss-of-function allele in UBA5 underlies a severe infantile-onset encephalopathy PMID: 27545674
Biallelic Variants in UBA5 Reveal that Disruption of the UFM1 Cascade Can Result in Early-Onset Encephalopathy PMID: 27545681
results of an in vitro truncation assay suggest that Uba5 residues 57-363 comprise the minimal fragment required for the high-efficiency activation of Ufm1 PMID: 24915089
ASC1 is a target for ufmylation and that UFBP1 is an essential component for ASC1 ufmylation. PMID: 25219498
binding of ATP to Uba5 approximately Ufm1 thioester was required for efficient transfer of Ufm1 from Uba5 to Ufc1 via transthiolation. PMID: 24966333
Thus, UfSP2 proteins have protease- and Ufm1-independent functions in GPCR biogenesis. PMID: 24603482
the Ufm1 system plays a critical role in vesicle trafficking, and its inhibition may lead to protein overload in the ER and UPR activation. PMID: 23152784
A novel type of E3 ligase for the Ufm1 conjugation system. PMID: 20018847
The solution structure and dynamics of human Ufm1 (hsUfm1) by nuclear magnetic resonance spectroscopy was analysed. PMID: 16527251