Literature DB >> 9228977

Conformational disease.

R W Carrell1, D A Lomas.   

Abstract

Several diverse disorders, including the prevalent dementias and encephalopathies, are now believed to arise from the same general disease mechanism. In each, there is abnormal unfolding and then aggregation of an underlying protein. The gradual accumulation of these aggregates and the acceleration of their formation by stress explain the characteristic late or episodic onset of the clinical disease. The understanding of these processes at the molecular level is opening prospects of more rational approaches to investigation and therapy.

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Year:  1997        PMID: 9228977     DOI: 10.1016/S0140-6736(97)02073-4

Source DB:  PubMed          Journal:  Lancet        ISSN: 0140-6736            Impact factor:   79.321


  204 in total

1.  Somatic mutations of the L12a gene in V-kappa(1) light chain deposition disease: potential effects on aberrant protein conformation and deposition.

Authors:  R Vidal; F Goñi; F Stevens; P Aucouturier; A Kumar; B Frangione; J Ghiso; G Gallo
Journal:  Am J Pathol       Date:  1999-12       Impact factor: 4.307

2.  Amyloid protofilament formation of hen egg lysozyme in highly concentrated ethanol solution.

Authors:  S Goda; K Takano; Y Yamagata; R Nagata; H Akutsu; S Maki; K Namba; K Yutani
Journal:  Protein Sci       Date:  2000-02       Impact factor: 6.725

Review 3.  Defective folding and rapid degradation of mutant proteins is a common disease mechanism in genetic disorders.

Authors:  N Gregersen; P Bross; M M Jørgensen; T J Corydon; B S Andresen
Journal:  J Inherit Metab Dis       Date:  2000-07       Impact factor: 4.982

4.  Reversible formation of on-pathway macroscopic aggregates during the folding of maltose binding protein.

Authors:  C Ganesh; F N Zaidi; J B Udgaonkar; R Varadarajan
Journal:  Protein Sci       Date:  2001-08       Impact factor: 6.725

Review 5.  Proteotoxicity in the endoplasmic reticulum: lessons from the Akita diabetic mouse.

Authors:  David Ron
Journal:  J Clin Invest       Date:  2002-02       Impact factor: 14.808

Review 6.  The role of chaperone-assisted folding and quality control in inborn errors of metabolism: protein folding disorders.

Authors:  N Gregersen; P Bross; B S Andrese; C B Pedersen; T J Corydon; L Bolund
Journal:  J Inherit Metab Dis       Date:  2001-04       Impact factor: 4.982

7.  Mutations in the B1 domain of protein G that delay the onset of amyloid fibril formation in vitro.

Authors:  Marina Ramírez-Alvarado; Melanie J Cocco; Lynne Regan
Journal:  Protein Sci       Date:  2003-03       Impact factor: 6.725

Review 8.  Liver injury in alpha1-antitrypsin deficiency: an aggregated protein induces mitochondrial injury.

Authors:  David H Perlmutter
Journal:  J Clin Invest       Date:  2002-12       Impact factor: 14.808

Review 9.  Translational control in the endoplasmic reticulum stress response.

Authors:  David Ron
Journal:  J Clin Invest       Date:  2002-11       Impact factor: 14.808

10.  Association between clinical characteristics and AL amyloid deposition in the kidney.

Authors:  Mitsuyo Itabashi; Takashi Takei; Misao Tsukada; Hidekazu Sugiura; Keiko Uchida; Ken Tsuchiya; Kazuho Honda; Kosaku Nitta
Journal:  Heart Vessels       Date:  2010-10-05       Impact factor: 2.037

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