The peripheral neuropathy-linked Trembler and Trembler-J mutant forms of peripheral myelin protein 22 are folding-destabilized.
about
Myelin-specific proteins: a structurally diverse group of membrane-interacting molecules.The homology model of PMP22 suggests mutations resulting in peripheral neuropathy disrupt transmembrane helix packing.The PMP22 gene and its related diseases.Inducible HSP70 is critical in preventing the aggregation and enhancing the processing of PMP22.Mutations associated with Charcot-Marie-Tooth disease cause SIMPLE protein mislocalization and degradation by the proteasome and aggresome-autophagy pathways.Protein misfolding and clearance in demyelinating peripheral neuropathies: Therapeutic implications.The safety dance: biophysics of membrane protein folding and misfolding in a cellular contextReversible folding of human peripheral myelin protein 22, a tetraspan membrane protein.Peripheral myelin protein 22 alters membrane architecture.Conformational Stability and Pathogenic Misfolding of the Integral Membrane Protein PMP22.Structural basis for the Trembler-J phenotype of Charcot-Marie-Tooth disease.The impact of window functions on NMR-based paramagnetic relaxation enhancement measurements in membrane proteins.Recent Advances in the Application of Solution NMR Spectroscopy to Multi-Span Integral Membrane Proteins.Influence of Pathogenic Mutations on the Energetics of Translocon-Mediated Bilayer Integration of Transmembrane Helices.
P2860
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P2860
The peripheral neuropathy-linked Trembler and Trembler-J mutant forms of peripheral myelin protein 22 are folding-destabilized.
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
2008年论文
@zh
2008年论文
@zh-cn
name
The peripheral neuropathy-link ...... n 22 are folding-destabilized.
@en
type
label
The peripheral neuropathy-link ...... n 22 are folding-destabilized.
@en
prefLabel
The peripheral neuropathy-link ...... n 22 are folding-destabilized.
@en
P2093
P2860
P356
P1433
P1476
The peripheral neuropathy-link ...... n 22 are folding-destabilized.
@en
P2093
Charles K Mobley
Charles R Sanders
Jeffrey K Myers
P2860
P304
10620-10629
P356
10.1021/BI801157P
P407
P577
2008-09-17T00:00:00Z