The endoplasmic reticulum degradation pathway for mutant secretory proteins alpha1-antitrypsin Z and S is distinct from that for an unassembled membrane protein.
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Deficient and Null Variants of SERPINA1 Are Proteotoxic in a Caenorhabditis elegans Model of α1-Antitrypsin DeficiencyAn HRD/DER-independent ER quality control mechanism involves Rsp5p-dependent ubiquitination and ER-Golgi transportMutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulumAlpha 1 antitrypsin deficiency in infants with neonatal cholestasisOligosaccharide modification in the early secretory pathway directs the selection of a misfolded glycoprotein for degradation by the proteasome.Alpha1-antitrypsin deficiency. 4: Molecular pathophysiologyActivating transcription factor 6 limits intracellular accumulation of mutant α(1)-antitrypsin Z and mitochondrial damage in hepatoma cells.Diagnosis and management of patients with α1-antitrypsin (A1AT) deficiency.Protein quality control: the who's who, the where's and therapeutic escapes.Endoplasmic reticulum quality control of asialoglycoprotein receptor H2a involves a determinant for retention and not retrieval.The Lhs1/GRP170 chaperones facilitate the endoplasmic reticulum-associated degradation of the epithelial sodium channelRapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse modelSingle nucleotide polymorphism-mediated translational suppression of endoplasmic reticulum mannosidase I modifies the onset of end-stage liver disease in alpha1-antitrypsin deficiency.Antisense oligonucleotide treatment ameliorates alpha-1 antitrypsin-related liver disease in mice.Intracellular processing of alpha1-antitrypsin.Liver disease in alpha-1 antitrypsin deficiency: current understanding and future therapy.Advances in alpha-1-antitrypsin deficiency liver disease.Alpha-1 antitrypsin and liver disease: mechanisms of injury and novel interventions.Endoplasmic reticulum quality control of unassembled iron transporter depends on Rer1p-mediated retrieval from the golgi.Role of ubiquitin in proteasomal degradation of mutant alpha(1)-antitrypsin Z in the endoplasmic reticulum.Disruption of disulfide bonds is responsible for impaired secretion in human complement factor H deficiency.A novel transcription factor regulates expression of the vacuolar H+-ATPase B2 subunit through AP-2 sites during monocytic differentiation.The final stage of gene expression: chaperones and the regulation of protein fate.Genetics and respiratory disease. 2. Alpha 1-antitrypsin deficiency, cirrhosis and emphysema.Mechanisms underlying the cellular clearance of antitrypsin Z: lessons from yeast expression systems.Degradation of human thyroperoxidase in the endoplasmic reticulum involves two different pathways depending on the folding state of the protein.Pathophysiology of Alpha-1 Antitrypsin Deficiency Liver Disease.Liver Disease in Alpha-1 Antitrypsin Deficiency: Current Approaches and Future Directions.Therapeutic Genome Editing With CRISPR/Cas9 in a Humanized Mouse Model Ameliorates α1-antitrypsin Deficiency Phenotype.
P2860
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P2860
The endoplasmic reticulum degradation pathway for mutant secretory proteins alpha1-antitrypsin Z and S is distinct from that for an unassembled membrane protein.
description
1996 nî lūn-bûn
@nan
1996年の論文
@ja
1996年論文
@yue
1996年論文
@zh-hant
1996年論文
@zh-hk
1996年論文
@zh-mo
1996年論文
@zh-tw
1996年论文
@wuu
1996年论文
@zh
1996年论文
@zh-cn
name
The endoplasmic reticulum degr ...... unassembled membrane protein.
@en
type
label
The endoplasmic reticulum degr ...... unassembled membrane protein.
@en
prefLabel
The endoplasmic reticulum degr ...... unassembled membrane protein.
@en
P2860
P356
P1476
The endoplasmic reticulum degr ...... unassembled membrane protein.
@en
P2093
Perlmutter DH
Teckman JH
P2860
P304
13215-13220
P356
10.1074/JBC.271.22.13215
P407
P577
1996-05-01T00:00:00Z