(Q40960179)

25 September 2017

title

Disruption of disulfide bonds is responsible for impaired secretion in human complement factor H deficiency (English)

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25 September 2017

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Schmidt BZ

1

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25 September 2017

Fowler NL

2

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25 September 2017

Hidvegi T

3

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25 September 2017

Perlmutter DH

4

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25 September 2017

Colten HR

5

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25 September 2017

language of work or name

English

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publication date

1 April 1999

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Journal of Biological Chemistry

25 September 2017

published in

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25 September 2017

volume

274

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25 September 2017

issue

17

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25 September 2017

page(s)

11782-11788

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Human factor H deficiency. Mutations in framework cysteine residues and block in H protein secretion and intracellular catabolism

25 September 2017

cites work

1 reference

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ISOLATION OF BETA IF-GLOBULIN FROM HUMAN SERUM AND ITS CHARACTERIZATION AS THE FIFTH COMPONENT OF COMPLEMENT.

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Interactions between human complement components factor H, factor I and C3b

21 January 2018

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Identification of human complement factor H as a chemotactic protein for monocytes.

21 January 2018

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Identification of three physically and functionally distinct binding sites for C3b in human complement factor H by deletion mutagenesis

21 January 2018

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Complement factor H and related proteins: an expanding family of complement-regulatory proteins?

21 January 2018

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The complete amino acid sequence of human complement factor H

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21 January 2018

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Solution structure of a pair of complement modules by nuclear magnetic resonance

21 January 2018

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Hereditary porcine membranoproliferative glomerulonephritis type II is caused by factor H deficiency

21 January 2018

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Familial hemolytic-uremic syndrome and homozygous factor H deficiency

21 January 2018

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Inherited factor H deficiency and collagen type III glomerulopathy.

21 January 2018

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Human complement factor H: isolation of cDNA clones and partial cDNA sequence of the 38-kDa tryptic fragment containing the binding site for C3b.

21 January 2018

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Lectin-binding sites as markers of Golgi subcompartments: proximal-to-distal maturation of oligosaccharides

21 January 2018

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Three new polymorphisms in the human complement factor H gene and promoter region

21 January 2018

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Disulfide bond formation during the folding of influenza virus hemagglutinin

21 January 2018

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Intracellular transport blockade caused by disruption of the disulfide bridge in the third external domain of major histocompatibility complex class I antigen

21 January 2018

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Functional analysis of disulfide linkages clustered within the amino terminus of human apolipoprotein B.

21 January 2018

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Accelerated secretion of human lysozyme with a disulfide bond mutation

21 January 2018

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Enhanced folding and processing of a disulfide mutant of the human asialoglycoprotein receptor H2b subunit

21 January 2018

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The efficiency of cysteine-mediated intracellular retention determines the differential fate of secretory IgA and IgM in B and plasma cells

21 January 2018

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Localization of the disulfide bond involved in post-translational processing of glycosylasparaginase and disrupted by a mutation in the Finnish-type aspartylglycosaminuria

21 January 2018

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Molecular and cellular basis of deficiency of the b subunit for factor XIII secondary to a Cys430-Phe mutation in the seventh Sushi domain

21 January 2018

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A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency

21 January 2018

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The endoplasmic reticulum degradation pathway for mutant secretory proteins alpha1-antitrypsin Z and S is distinct from that for an unassembled membrane protein

21 January 2018

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Type II human complement C2 deficiency. Allele-specific amino acid substitutions (Ser189 --> Phe; Gly444 --> Arg) cause impaired C2 secretion

21 January 2018

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Degradation of CFTR by the ubiquitin-proteasome pathway

21 January 2018

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Carboxy terminally truncated forms of ribophorin I are degraded in pre-Golgi compartments by a calcium-dependent process

21 January 2018

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Role of 26S proteasome and HRD genes in the degradation of 3-hydroxy-3-methylglutaryl-CoA reductase, an integral endoplasmic reticulum membrane protein

21 January 2018

1 reference

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21 January 2018

Identifiers

DOI

10.1074/JBC.274.17.11782

1 reference

25 September 2017

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