GENETIC PREDISPOSITION TO IATROGENIC CREUTZFELDT-JAKOB DISEASE

被引：471

作者：

COLLINGE, J

PALMER, MS

DRYDEN, AJ

机构：

[1] Prion Disease Group, Department of Biochemistry and Molecular Genetics, St Mary's Hospital Medical School, London, W2 1PG, Norfolk Place

来源：

LANCET | 1991年 / 337卷 / 8755期

基金：

英国惠康基金;

关键词：

D O I：

10.1016/0140-6736(91)93128-V

中图分类号：

R5 [内科学];

学科分类号：

1002 [临床医学]; 100201 [内科学];

摘要：

The spongiform encephalopathy Creutzfeldt-Jakob disease (CJD) has been transmitted to man via administration of growth hormone and gonadotropin extracted from large pooled batches of human cadaveric pituitary glands. In the UK, 1908 individuals were exposed to potentially contaminated growth hormone, of whom 6 have so far manifested CJD. Examination of the prion protein genes of all these cases and of a single case of gonadotropin-related CJD showed that 4 had the uncommon valine 129 homozygous genotype indicating genetic susceptibility to prion infection. Such genetic susceptibility may be important in the aetiology of sporadic CJD disease.

引用

页码：1441 / 1442

页数：2

共 6 条

[1]

MORTALITY, NEOPLASIA, AND CREUTZFELDT-JAKOB DISEASE IN PATIENTS TREATED WITH HUMAN PITUITARY GROWTH-HORMONE IN THE UNITED-KINGDOM [J].

BUCHANAN, CR ;

PREECE, MA ;

MILNER, RDG .

BRITISH MEDICAL JOURNAL, 1991, 302 (6780) :824-828

[2]

DIAGNOSIS OF GERSTMANN-STRAUSSLER SYNDROME IN FAMILIAL DEMENTIA WITH PRION PROTEIN GENE ANALYSIS [J].

COLLINGE, J ;

HARDING, AE ;

OWEN, F ;

POULTER, M ;

LOFTHOUSE, R ;

BOUGHEY, AM ;

SHAH, T ;

CROW, TJ .

LANCET, 1989, 2 (8653) :15-17

[3]

GOLDFARB L G, 1989, American Journal of Human Genetics, V45, pA189

[4]

SPONGIFORM ENCEPHALOPATHIES - THE PRIONS PROGRESS [J].

WEISSMANN, C .

NATURE, 1991, 349 (6310) :569-571

[5]

DISTINCT PRION PROTEINS IN SHORT AND LONG SCRAPIE INCUBATION PERIOD MICE [J].

WESTAWAY, D ;

GOODMAN, PA ;

MIRENDA, CA ;

MCKINLEY, MP ;

CARLSON, GA ;

PRUSINER, SB .

CELL, 1987, 51 (04) :651-662

[6]

A PVUII RFLP DETECTED IN THE HUMAN PRION PROTEIN (PRP) GENE [J].

WU, Y ;

BROWN, WT ;

ROBAKIS, NK ;

DOBKIN, C ;

DEVINEGAGE, E ;

MERZ, P ;

WISNIEWSKI, HM .

NUCLEIC ACIDS RESEARCH, 1987, 15 (07) :3191-3191

← 1 →