INHIBITION OF TAMM-HORSFALL GLYCOPROTEIN INDUCTION OF ALKALINE-PHOSPHATASE IN CYSTIC-FIBROSIS FIBROBLASTS BY MEDIUM CONDITIONED BY NORMAL-CELLS

被引：5

作者：

CAREY, WF ^{[1
]}

HOSLI, P ^{[1
]}

机构：

[1] INST PASTEUR,DEPT BIOL MOLEC,UNITE CYTOGENET EXPTL,F-75015 PARIS,FRANCE

来源：

AUSTRALIAN JOURNAL OF EXPERIMENTAL BIOLOGY AND MEDICAL SCIENCE | 1979年 / 57卷 / APR期

关键词：

D O I：

10.1038/icb.1979.23

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

摘要：

It is confirmed that the level of alkaline phosphatase in fibroblasts derived from cystic fibrosis patients can be induced many-fold by growing the cells in the presence of Tamm-Horsfall glycoprotein. It is further shown that normal fibroblasts produce a 'CF corrective factor' which markedly inhibits this phenomenon. These observations support a previous hypothesis on the nature of the metabolic defect in cystic fibrosis.

引用

页码：225 / 230

页数：6

共 10 条

[1] DEFECT IN HURLER AND SCHEIE SYNDROMES - DEFICIENCY OF ALPHA-IDURONIDASE [J].

BACH, G ;

WEISSMAN.B ;

FRIEDMAN, R ;

NEUFELD, EF .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1972, 69 (08) :2048-&

[2] DEFECT IN HURLER AND HUNTER SYNDROMES, .2. DEFICIENCY OF SPECIFIC FACTORS INVOLVED IN MUCOPOLYSACCHARIDE DEGRADATION [J].

FRATANTO.JC ;

HALL, CW ;

NEUFELD, EF .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1969, 64 (01) :360-&

[3]

Hosli P, 1976, Adv Exp Med Biol, V68, P1

[4]

HOSLI P, 1978, HUM GENET, V41, P169

[5] RELIABLE DIAGNOSIS OF MAJOR TYPE OF CYSTIC-FIBROSIS WITH FIBROBLAST-CULTURES - DOUBLE-BLIND STUDY [J].

HOSLI, P ;

KOLLBERG, H ;

VOGT, E .

ACTA PAEDIATRICA SCANDINAVICA, 1978, 67 (05) :617-620

[6] CYSTIC-FIBROSIS - LEAKAGE OF LYSOSOMAL ENZYMES AND OF ALKALINE-PHOSPHATASE INTO EXTRACELLULAR-SPACE [J].

HOSLI, P ;

VOGT, E .

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1977, 79 (03) :741-748

[7] PROSPECTS FOR PRENATAL DIAGNOSIS OF CYSTIC-FIBROSIS - INDUCTION OF BIOCHEMICAL ABNORMALITIES IN FIBROBLASTS FROM PATIENTS WITH CYSTIC-FIBROSIS BY A URINARY GLYCOPROTEIN [J].

HOSLI, P ;

ERICKSON, RP ;

VOGT, E .

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1976, 73 (02) :209-216

[8]

HOSLI P, 1977, CLIN CHEM, V23, P1476

[9]

HOSLI P, 1978, MONOGR PAEDIATR, V10, P21

[10]

HOSLI P, 1976, 7TH P INT CYST FIBR, P278

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