C-terminal mutations destabilize SIL1/BAP and can cause Marinesco-Sjögren syndrome.
about
The nucleotide exchange factors of Hsp70 molecular chaperonesBiP and its nucleotide exchange factors Grp170 and Sil1: mechanisms of action and biological functionsA nationwide survey on Marinesco-Sjögren syndrome in Japan.Enhancing human spermine synthase activity by engineered mutationsSil1, a nucleotide exchange factor for BiP, is not required for antibody assembly or secretionAn involvement of oxidative stress in endoplasmic reticulum stress and its associated diseases.The large Hsp70 Grp170 binds to unfolded protein substrates in vivo with a regulation distinct from conventional Hsp70s.SIL1, a causative cochaperone gene of Marinesco-Söjgren syndrome, plays an essential role in establishing the architecture of the developing cerebral cortexThe autosomal dominant spinocerebellar ataxias: emerging mechanistic themes suggest pervasive Purkinje cell vulnerability.Perspectives in Pediatric Pathology, Chapter 17. Other Hypergonadotropic Hypogonadisms.Sil1-Mutant Mice Elucidate Chaperone Function in Neurological Disorders.Cellular Signature of SIL1 Depletion: Disease Pathogenesis due to Alterations in Protein Composition Beyond the ER Machinery.SIL1 mutations and clinical spectrum in patients with Marinesco-Sjogren syndrome.Bap (Sil1) regulates the molecular chaperone BiP by coupling release of nucleotide and substrate.SIL1 Rescued Bip Elevation-Related Tau Hyperphosphorylation in ER Stress.Tracking Effects of SIL1 Increase: Taking a Closer Look Beyond the Consequences of Elevated Expression Level.
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P2860
C-terminal mutations destabilize SIL1/BAP and can cause Marinesco-Sjögren syndrome.
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
2012年论文
@zh
2012年论文
@zh-cn
name
C-terminal mutations destabilize SIL1/BAP and can cause Marinesco-Sjögren syndrome.
@ast
C-terminal mutations destabilize SIL1/BAP and can cause Marinesco-Sjögren syndrome.
@en
type
label
C-terminal mutations destabilize SIL1/BAP and can cause Marinesco-Sjögren syndrome.
@ast
C-terminal mutations destabilize SIL1/BAP and can cause Marinesco-Sjögren syndrome.
@en
prefLabel
C-terminal mutations destabilize SIL1/BAP and can cause Marinesco-Sjögren syndrome.
@ast
C-terminal mutations destabilize SIL1/BAP and can cause Marinesco-Sjögren syndrome.
@en
P2093
P2860
P356
P1476
C-terminal mutations destabilize SIL1/BAP and can cause Marinesco-Sjögren syndrome.
@en
P2093
Jennifer Howes
Linda M Hendershot
Matthias J Feige
Yuichiro Shimizu
P2860
P304
P356
10.1074/JBC.M111.333286
P407
P577
2012-01-04T00:00:00Z