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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
3
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pubmed:dateCreated |
1981-10-14
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pubmed:abstractText |
Erythrocytes of many patients with pseudohypoparathyroidism, type I (PHP-I), exhibit reduced activity of the N protein, a guanine nucleotide-binding regulatory component of hormone-sensitive adenylate cyclase. We compared N and adenylate cyclase activities and the accumulation of cAMP in fibroblasts propagated from skin biopsies of six normal subjects and seven PHP-I patients. N activities were reduced by approximately 40% in fibroblasts as well as erythrocytes of five PHP-I patients. N activities in fibroblasts from two PHP-I patients with normal erythrocyte N activities were within the normal range. These results are consistent with the hypothesis that N deficiency is generalized in tissues of most PHP-I patients and is the primary defect responsible for their resistance to metabolic effects of hormones that work by stimulating adenylate cyclase. Fibroblast N deficiency was not associated with decreases in hormone-stimulated adenylate cyclase or cAMP accumulation in fibroblasts, probably because these activities involve many potentially regulable cellular components in addition to the N protein.
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pubmed:grant |
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
AIM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Sep
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pubmed:issn |
0021-972X
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:volume |
53
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
636-40
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pubmed:dateRevised |
2007-11-14
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pubmed:meshHeading |
pubmed-meshheading:6267099-Adenylate Cyclase,
pubmed-meshheading:6267099-Adolescent,
pubmed-meshheading:6267099-Adult,
pubmed-meshheading:6267099-Cells, Cultured,
pubmed-meshheading:6267099-Cyclic AMP,
pubmed-meshheading:6267099-Erythrocytes,
pubmed-meshheading:6267099-Female,
pubmed-meshheading:6267099-Fibroblasts,
pubmed-meshheading:6267099-GTP-Binding Proteins,
pubmed-meshheading:6267099-Guanine Nucleotides,
pubmed-meshheading:6267099-Humans,
pubmed-meshheading:6267099-Kinetics,
pubmed-meshheading:6267099-Male,
pubmed-meshheading:6267099-Middle Aged,
pubmed-meshheading:6267099-Parathyroid Hormone,
pubmed-meshheading:6267099-Prostaglandins E,
pubmed-meshheading:6267099-Pseudohypoparathyroidism,
pubmed-meshheading:6267099-Receptors, Cell Surface,
pubmed-meshheading:6267099-Skin
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pubmed:year |
1981
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pubmed:articleTitle |
Fibroblast defect in pseudohypoparathyroidism, type I: reduced activity of receptor-cyclase coupling protein.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, U.S. Gov't, Non-P.H.S.
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