DEPLETION OF ALCOHOL (HEXANOL) DEHYDROGENASE-ACTIVITY IN THE EPIDERMIS AND JEJUNAL MUCOSA IN SJOGREN-LARSSON SYNDROME

被引：31

作者：

JUDGE, MR

LAKE, BD

SMITH, VV

BESLEY, GTN

HARPER, JI

机构：

[1] HOSP SICK CHILDREN,GREAT ORMOND ST,LONDON WC1N 3JH,ENGLAND

[2] INST CHILD HLTH,DEPT DERMATOL,LONDON WC1N 1EH,ENGLAND

[3] INST CHILD HLTH,DEPT HISTOPATHOL,LONDON WC1N 1EH,ENGLAND

[4] ROYAL INFIRM,DEPT PAEDIAT BIOCHEM,EDINBURGH EH3 9HB,MIDLOTHIAN,SCOTLAND

来源：

JOURNAL OF INVESTIGATIVE DERMATOLOGY | 1990年 / 95卷 / 06期

关键词：

D O I：

10.1111/1523-1747.ep12514294

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

Using a histochemical technique, we have demonstrated a consistent deficiency of alcohol (hexanol) dehydrogenase activity within the epidermis and jejunal mucosa of patients with Sjogren-Larsson syndrome. Biochemical assay of the fatty alcohol: NAD oxidoreductase activity in cultured fibroblasts and leukocytes from these patients showed deficient activities compared with controls. The histochemical and biochemical results are complementary, and the simpler histochemical method can be used reliably for initial screening of patients with ichthyosis in whom a diagnosis of Sjogren-Larsson syndrome is suspected.

引用

页码：632 / 634

页数：3

共 7 条

[1] STUDIES ON SPHINGOMYELINASE AND BETA-GLUCOSIDASE ACTIVITIES IN NIEMANN-PICK DISEASE VARIANTS - PHOSPHODIESTERASE ACTIVITIES MEASURED WITH NATURAL AND ARTIFICIAL SUBSTRATES [J].