Molecular abnormality of human alpha1-antitrypsin variant (Pi-ZZ) associated with plasma activity deficiency
about
Challenges and Prospects for Alpha-1 Antitrypsin Deficiency Gene TherapyBiochemical characterization of a neuroserpin variant associated with hereditary dementiaImmunopathology of the lung: a reviewGenetic studies on a new deficiency gene (PI*Ztun) at the PI locus.Environmental and genetic risk factors and gene-environment interactions in the pathogenesis of chronic obstructive lung diseaseEvaluation of "at risk" alpha 1-antitrypsin genotype SZ with synthetic oligonucleotide gene probesSynthesis of stress proteins is increased in individuals with homozygous PiZZ alpha 1-antitrypsin deficiency and liver diseaseMolecular analysis of the gene of the alpha 1-antitrypsin deficiency variant, MnichinanComplete absence of serum alpha-1-antitrypsin in conjunction with an apparently normal gene structureAlpha 1-antitrypsin: apparent molecular weight heterogeneity shown by two-dimensional electrophoresis.Molecular abnormality of PI S variant of human alpha1-antitrypsinAssignment of the alpha 1-antitrypsin gene and a sequence-related gene to human chromosome 14 by molecular hybridizationAn alpha 1-antitrypsin variant, Pi B Alhambra (Lys to Asp, Glu to Asp), with rapid anodal electrophoretic mobility.Structural difference between the normal PiM1 and the common PiM2 variant of human alpha 1-antitrypsin.Z-type alpha 1-antitrypsin is less competent than M1-type alpha 1-antitrypsin as an inhibitor of neutrophil elastase.Alpha1-antitrypsin deficiency. 4: Molecular pathophysiologyTissue specific expression of the human alpha-1-antitrypsin gene in transgenic mice.Impaired hepcidin expression in alpha-1-antitrypsin deficiency associated with iron overload and progressive liver disease.Mechanisms for the incorporation of proteins in membranes and organelles.Mutations of the Rous sarcoma virus env gene that affect the transport and subcellular location of the glycoprotein products.Assignment of human alpha 1-antitrypsin to chromosome 14 by somatic cell hybrid analysisCloning and sequence of cDNA coding for alpha 1-antitrypsin.A defect in the structure of type I procollagen in a patient who had osteogenesis imperfecta: excess mannose in the COOH-terminal propeptide.Synthesis of abnormal immunoglobulins by hybridomas from autoimmune "viable motheaten" mutant mice.What we owe to alpha(1)-antitrypsin and to Carl-Bertil Laurell.Molecular basis for defective secretion of the Z variant of human alpha-1-proteinase inhibitor: secretion of variants having altered potential for salt bridge formation between amino acids 290 and 342.Expression of the alpha-1-antitrypsin gene in mononuclear phagocytes of normal and alpha-1-antitrypsin-deficient individualsReplacement therapy of alpha 1-antitrypsin deficiency. Reversal of protease-antiprotease imbalance within the alveolar structures of PiZ subjectsDanazol-induced augmentation of serum alpha 1-antitrypsin levels in individuals with marked deficiency of this antiprotease.The cellular defect in alpha 1-proteinase inhibitor (alpha 1-PI) deficiency is expressed in human monocytes and in Xenopus oocytes injected with human liver mRNA.A null deficiency allele of alpha 1-antitrypsin, QOludwigshafen, with altered tertiary structure.alpha-1-Antitrypsin: sequence of the Z variant tryptic peptide.Alpha-1-Antitrysin: structural relationships of the substitutions of the S and Z variants.Elastase regulates the synthesis of its inhibitor, alpha 1-proteinase inhibitor, and exaggerates the defect in homozygous PiZZ alpha 1 PI deficiency.The biostructural pathology of the serpins: critical function of sheet opening mechanism.BiP expression is not increased by the accumulation of PiZ alpha 1-antitrypsin in the endoplasmic reticulum.Posttranslational association of immunoglobulin heavy chain binding protein with nascent heavy chains in nonsecreting and secreting hybridomas.Review: alpha 1-antitrypsin deficiency associated liver disease.Genetics and respiratory disease. 2. Alpha 1-antitrypsin deficiency, cirrhosis and emphysema.Polymers of alpha(1)-antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema.
P2860
Q28080450-EC1F8C6E-0B5A-4C49-B28F-EF37496756ECQ28345664-BFB43918-2B74-4BD5-AE1B-52B6927530C0Q28397007-DE922FF0-6514-42E4-8DD5-A915E0D5F1A7Q33593417-88615D8A-9817-48EC-A701-718C3480CAB5Q33994809-824872CD-0B3C-41CB-835F-446A0654CEFFQ34521234-717BA2A4-D891-4474-B2BF-C79FFBA7EB29Q34579205-F4135E19-F461-4DDB-8A5F-E6F7C1C78515Q35197886-AFFD23BE-2449-42F1-B203-83B740E546A4Q35200981-DB7D33E1-8230-4EBA-ACDE-883D12F5F07DQ35202083-FBF7DBC3-BA01-4BF8-BF2E-EFCB249AE634Q35202173-4D841E89-44E3-4D16-96A4-CD3D6DA02DBAQ35202850-8B6CBE9E-530A-4A35-85DC-FF69867DDA4BQ35203466-C2287022-A12D-443E-BBF9-78D4CCDCC562Q35203469-8ED7F8A6-80AC-4507-8998-D10C689B6013Q35581750-0A2F7B76-D375-4106-881C-D7F0D49A3656Q35788002-CEF3468E-E12D-4441-AE09-3C3F3BB13FC7Q36118723-52E999AE-DD0C-44D7-9445-74F35E13A7B6Q36142416-D96D5D2F-0AD2-4299-8E25-B3CA03C4705CQ36205692-79851B06-4679-4E33-BEA9-7DC05A412C88Q36212065-FA3344A8-6579-4301-8FD2-667D078F8C7FQ36280639-78F93455-6A4D-4DFB-A87D-D37547B4EE33Q36378217-20E50564-AF8A-48C9-86CA-5AE01AA23F59Q36412261-28939BA5-0C58-473E-8686-10045B2FEBAEQ36529975-23F3A6FB-071D-43A7-9CF0-05CDE57D04B5Q36602296-DB57A797-AD33-44C7-98D8-CBB2AD89C95EQ36763414-2137F51A-C199-45DB-A84D-339E411AE11EQ36994663-344D80C8-3F64-4C24-9A38-32EAE0A9A77CQ37004889-195B3DFD-19F2-4C10-BFA1-1E7A0EC0AA38Q37023792-DE68B185-05DF-4E57-B69F-A1F4DD4906FFQ37536001-BF14DFF8-FF36-448B-8484-0A9AED11F38EQ40511861-8375487C-E4B5-41F3-B48D-D5B6740883B6Q40653898-7E6923D0-956A-4ED2-84CC-4E59AE0309E8Q40676482-E457E1D3-8460-48AB-9115-E21B168C3EC4Q41017471-B8DC3284-4FA7-4AE9-AC60-9549AE5E03E4Q41224616-AF94D509-FBC4-456D-BD71-B05EAD15A16CQ41236581-F5A2545F-578C-4D08-A08D-3FD659605AB3Q41506259-0884CD7E-9D4E-4BE3-8E72-D0D3857C450BQ41509050-9D987134-4E25-4B83-BAA0-87836CFAD7A7Q41807676-192285E6-59FA-46F7-A183-387F17B75FE7Q42521815-8CB40829-BBC7-4185-B1B6-4229307F515F
P2860
Molecular abnormality of human alpha1-antitrypsin variant (Pi-ZZ) associated with plasma activity deficiency
description
1976 nî lūn-bûn
@nan
1976 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
1976 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
1976年の論文
@ja
1976年論文
@yue
1976年論文
@zh-hant
1976年論文
@zh-hk
1976年論文
@zh-mo
1976年論文
@zh-tw
1976年论文
@wuu
name
Molecular abnormality of human ...... ith plasma activity deficiency
@ast
Molecular abnormality of human ...... ith plasma activity deficiency
@en
Molecular abnormality of human alpha1-antitrypsin variant
@nl
type
label
Molecular abnormality of human ...... ith plasma activity deficiency
@ast
Molecular abnormality of human ...... ith plasma activity deficiency
@en
Molecular abnormality of human alpha1-antitrypsin variant
@nl
prefLabel
Molecular abnormality of human ...... ith plasma activity deficiency
@ast
Molecular abnormality of human ...... ith plasma activity deficiency
@en
Molecular abnormality of human alpha1-antitrypsin variant
@nl
P2093
P2860
P356
P1476
Molecular abnormality of human ...... ith plasma activity deficiency
@en
P2093
P2860
P304
P356
10.1073/PNAS.73.4.1324
P407
P577
1976-04-01T00:00:00Z