A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency.
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Human factor H deficiency. Mutations in framework cysteine residues and block in H protein secretion and intracellular catabolismCharacterization of an ERAD gene as VPS30/ATG6 reveals two alternative and functionally distinct protein quality control pathways: one for soluble Z variant of human alpha-1 proteinase inhibitor (A1PiZ) and another for aggregates of A1PiZ.Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapyLiver injury in alpha1-antitrypsin deficiency: an aggregated protein induces mitochondrial injurySmall molecules block the polymerization of Z alpha1-antitrypsin and increase the clearance of intracellular aggregatesLiver tumors in children with metabolic disordersThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyADD66, a gene involved in the endoplasmic reticulum-associated degradation of alpha-1-antitrypsin-Z in yeast, facilitates proteasome activity and assembly.Elucidation of the molecular logic by which misfolded alpha 1-antitrypsin is preferentially selected for degradation.Functional expression of the Wilson disease protein reveals mislocalization and impaired copper-dependent trafficking of the common H1069Q mutation.Modeling inherited metabolic disorders of the liver using human induced pluripotent stem cells.Protein misfolding and degradation in genetic diseases.Oligosaccharide modification in the early secretory pathway directs the selection of a misfolded glycoprotein for degradation by the proteasome.Z α1-antitrypsin confers a proinflammatory phenotype that contributes to chronic obstructive pulmonary diseaseOrganizational diversity among distinct glycoprotein endoplasmic reticulum-associated degradation programsIs heterozygous alpha-1-antitrypsin deficiency type PIZ a risk factor for primary liver carcinoma?Interaction of Hepatitis B virus with cellular processes in liver carcinogenesis.A C. elegans model of human α1-antitrypsin deficiency links components of the RNAi pathway to misfolded protein turnoverInherited liver diseases in adultsAlpha-1 antitrypsin deficiencyMechanisms of liver injury relevant to pediatric hepatology.Z α-1 antitrypsin deficiency and the endoplasmic reticulum stress response.Orchestrating the unfolded protein response in health and disease.Mutant fibrinogen cleared from the endoplasmic reticulum via endoplasmic reticulum-associated protein degradation and autophagy: an explanation for liver disease.Role of Drosophila EDEMs in the degradation of the alpha-1-antitrypsin Z variant.PiZ mouse liver accumulates polyubiquitin conjugates that associate with catalytically active 26S proteasomesThe Apo(a) gene is the major determinant of variation in plasma Lp(a) levels in African Americans.Alpha1-antitrypsin deficiency. 4: Molecular pathophysiologyIdentification of 13 new mutations in the vasopressin-neurophysin II gene in 17 kindreds with familial autosomal dominant neurohypophyseal diabetes insipidusDiagnosis and management of patients with α1-antitrypsin (A1AT) deficiency.Disrupted proteolipid protein trafficking results in oligodendrocyte apoptosis in an animal model of Pelizaeus-Merzbacher disease.Protein folding in the endoplasmic reticulum: lessons from the human chorionic gonadotropin beta subunitSVIP regulates Z variant alpha-1 antitrypsin retro-translocation by inhibiting ubiquitin ligase gp78.The endosomal protein-sorting receptor sortilin has a role in trafficking α-1 antitrypsinPathogenesis of chronic liver injury and hepatocellular carcinoma in alpha-1-antitrypsin deficiency.What we owe to alpha(1)-antitrypsin and to Carl-Bertil Laurell.The protective and destructive roles played by molecular chaperones during ERAD (endoplasmic-reticulum-associated degradation).Disorders of protein misfolding: alpha-1-antitrypsin deficiency as prototype.Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency.Rapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse model
P2860
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P2860
A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency.
description
1994 nî lūn-bûn
@nan
1994年の論文
@ja
1994年論文
@yue
1994年論文
@zh-hant
1994年論文
@zh-hk
1994年論文
@zh-mo
1994年論文
@zh-tw
1994年论文
@wuu
1994年论文
@zh
1994年论文
@zh-cn
name
A lag in intracellular degrada ...... lpha 1-antitrypsin deficiency.
@ast
A lag in intracellular degrada ...... lpha 1-antitrypsin deficiency.
@en
type
label
A lag in intracellular degrada ...... lpha 1-antitrypsin deficiency.
@ast
A lag in intracellular degrada ...... lpha 1-antitrypsin deficiency.
@en
prefLabel
A lag in intracellular degrada ...... lpha 1-antitrypsin deficiency.
@ast
A lag in intracellular degrada ...... lpha 1-antitrypsin deficiency.
@en
P2093
P2860
P356
P1476
A lag in intracellular degrada ...... lpha 1-antitrypsin deficiency.
@en
P2093
P2860
P304
P356
10.1073/PNAS.91.19.9014
P407
P577
1994-09-01T00:00:00Z