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UFM1 is a ubiquitin-like protein that is conjugated to target proteins by E1-like activating enzyme UBA5 (UBE1DC1\; MIM 610552) and E2-like conjugating enzyme UFC1 (MIM 610554) in a manner analogous to ubiquitylation (see UBE2M\; MIM 603173) (Komatsu et al., 2004 [PubMed 15071506]).[supplied by OMIM, Dec 2008].. Zusätzlich bieten wir Ihnen UFM1 Antikörper (12) und und viele weitere Produktgruppen zu diesem Protein an.
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UFM1 His 70 resembles UBA5 (zeige UBA5 Proteine) His336 and enters a negatively charged pocked on the other UFM1 molecule.
These findings explicitly elucidate the role of UBA5 (zeige UBA5 Proteine) dimerization in UFM1 activation.
This study, focused on identifying the mutated gene in patients with hypomyelination with atrophy of the basal ganglia and cerebellum without TUBB4A (zeige TUBB5 Proteine) mutations, revealed a homozygous 3-bp deletion in the UFM1 promoter area, which perfectly segregates with the disease.
Meta analysis identified a new gene, UFM1, that is associated with low von Willebrand factor (zeige VWF Proteine) levels.
in the absence of UFSP2 (zeige C4orf20 Proteine), a deconjugating enzyme for UFM1, ectopic expression of both UFL1 (zeige UFL1 Proteine) and UFBP1, which serve as the E3-ligase complex for the UFM1-system, dramatically increases UFM1-conjugate formation at the endoplasmic reticulum.
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
Biallelic Variants in UBA5 (zeige UBA5 Proteine) Reveal that Disruption of the UFM1 Cascade Can Result in Early-Onset Encephalopathy
results of an in vitro truncation assay suggest that Uba5 (zeige UBA5 Proteine) residues 57-363 comprise the minimal fragment required for the high-efficiency activation of Ufm1
ASC1 (zeige RPL3 Proteine) is a target for ufmylation and that UFBP1 is an essential component for ASC1 (zeige RPL3 Proteine) ufmylation.
binding of ATP to Uba5 (zeige UBA5 Proteine) approximately Ufm1 thioester was required for efficient transfer of Ufm1 from Uba5 (zeige UBA5 Proteine) to Ufc1 (zeige UFC1 Proteine) via transthiolation.
CNS-specific knockout of Ufm1 in mice causes neonatal death accompanied by microcephaly and apoptosis in specific neurons, further suggesting that the UFM1 system is essential for CNS development and function
High UFM1 expression is associated with Atherosclerosis.
transcription downregulation of the Ufm1 and FAT10 (zeige UBD Proteine) conjugation system in liver Mallory-Denk bodies formation
These data suggest that UFM1-UFBP1 participate in preventing ER stress-induced apoptosis in protein secretory cells.
A novel type of E3 ligase for the Ufm1 conjugation system.
A novel ubiquitin fold modifier (Ufm1) not been previously associated with ER stress and not found to be induced under any condition was found to be upregulated in the hearts of MCP (zeige CR1L Proteine) mice (transgenic mice that express MCP-1 (zeige CPT1B Proteine) specifically in the heart).
loop between beta3 and alpha2 in addition to the C-terminal region of Ufm1 plays a role in binding to UfSP1
UFM1 is a ubiquitin-like protein that is conjugated to target proteins by E1-like activating enzyme UBA5 (UBE1DC1\; MIM 610552) and E2-like conjugating enzyme UFC1 (MIM 610554) in a manner analogous to ubiquitylation (see UBE2M\; MIM 603173) (Komatsu et al., 2004
ubiquitin-fold modifier 1