Multistep, sequential control of the trafficking and function of the multiple sulfatase deficiency gene product, SUMF1 by PDI, ERGIC-53 and ERp44.
about
Crystal structure of the LMAN1-CRD/MCFD2 transport receptor complex provides insight into combined deficiency of factor V and factor VIIIEfficacy of a combined intracerebral and systemic gene delivery approach for the treatment of a severe lysosomal storage disorder.Puzzle of protein complexes in vivo: a present and future challenge for functional proteomics.The ribosomal protein L2 interacts with the RNA polymerase alpha subunit and acts as a transcription modulator in Escherichia coli.Progressive quality control of secretory proteins in the early secretory compartment by ERp44Natural disease history and characterisation of SUMF1 molecular defects in ten unrelated patients with multiple sulfatase deficiencyGolgi and related vesicle proteomics: simplify to identify.Phenylbutyric acid suppresses protein accumulation-mediated ER stress in retrovirus-infected astrocytes and delays onset of paralysis in infected miceERp44 C160S/C212S mutants regulate IP3R1 channel activityERP44 inhibits human lung cancer cell migration mainly via IP3R2Dynamic regulation of Ero1α and peroxiredoxin 4 localization in the secretory pathway.Cell biology of the endoplasmic reticulum and the Golgi apparatus through proteomicsMissing links in antibody assembly control.Formylglycine, a post-translationally generated residue with unique catalytic capabilities and biotechnology applications.Aberrant disulphide bonding contributes to the ER retention of alpha1-antitrypsin deficiency variants.Proprotein convertases process and thereby inactivate formylglycine-generating enzyme.A pH-regulated quality control cycle for surveillance of secretory protein assembly.Sulfatase activities are regulated by the interaction of the sulfatase-modifying factor 1 with SUMF2.Molecular bases of cyclic and specific disulfide interchange between human ERO1alpha protein and protein-disulfide isomerase (PDI).SUMF1 mutations affecting stability and activity of formylglycine generating enzyme predict clinical outcome in multiple sulfatase deficiency.Crystal and solution structures of human protein disulfide isomerase-like protein of the testis (PDILT) provide insight into its chaperone activity.Case of multiple sulfatase deficiency and ocular albinism: a diagnostic odyssey.Eukaryotic formylglycine-generating enzyme catalyses a monooxygenase type of reaction.
P2860
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P2860
Multistep, sequential control of the trafficking and function of the multiple sulfatase deficiency gene product, SUMF1 by PDI, ERGIC-53 and ERp44.
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年学术文章
@wuu
2008年学术文章
@zh
2008年学术文章
@zh-cn
2008年学术文章
@zh-hans
2008年学术文章
@zh-my
2008年学术文章
@zh-sg
2008年學術文章
@yue
2008年學術文章
@zh-hant
name
Multistep, sequential control ...... F1 by PDI, ERGIC-53 and ERp44.
@en
Multistep, sequential control ...... F1 by PDI, ERGIC-53 and ERp44.
@nl
type
label
Multistep, sequential control ...... F1 by PDI, ERGIC-53 and ERp44.
@en
Multistep, sequential control ...... F1 by PDI, ERGIC-53 and ERp44.
@nl
prefLabel
Multistep, sequential control ...... F1 by PDI, ERGIC-53 and ERp44.
@en
Multistep, sequential control ...... F1 by PDI, ERGIC-53 and ERp44.
@nl
P2093
P50
P356
P1476
Multistep, sequential control ...... F1 by PDI, ERGIC-53 and ERp44.
@en
P2093
Alessandro Fraldi
Carmine Spampanato
Ester Zito
Marianna Cozzolino
Piero Pucci
P304
P356
10.1093/HMG/DDN161
P577
2008-05-28T00:00:00Z