RGD Reference Report - Metabolism of 25-hydroxyvitamin D in copper-laden rat: a model of Wilson's disease. - Rat Genome Database

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Metabolism of 25-hydroxyvitamin D in copper-laden rat: a model of Wilson's disease.

Authors: Carpenter, TO  Pendrak, ML  Anast, CS 
Citation: Carpenter TO, etal., Am J Physiol. 1988 Feb;254(2 Pt 1):E150-4.
RGD ID: 2307322
Pubmed: PMID:3348368   (View Abstract at PubMed)

Wilson's disease results in excess tissue accumulation of copper and is often complicated by skeletal and mineral abnormalities. We investigated vitamin D metabolism in rats fed a copper-laden diet rendering hepatic copper content comparable with that found in Wilson's disease. Injection of 25-hydroxyvitamin D3 [25(OH)D3] resulted in reduced 1,25-dihydroxyvitamin D [1,25(OH)2D] levels in copper-intoxicated rats. In vitro 25(OH)D-1 alpha-hydroxylase activity was impaired in renal mitochondria from copper-intoxicated animals. Activity was also inhibited in mitochondria from controls when copper was added to incubation media. Impaired conversion of 25(OH)D to 1,25(OH)2D occurs in copper intoxication and suggests that altered vitamin D metabolism is a potential factor in the development of bone and mineral abnormalities in Wilson's disease.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
Wilson disease  ISOCyp27b1 (Rattus norvegicus)2307322; 2307322 RGD 
Wilson disease  IDA 2307322 RGD 

Gene Ontology Annotations    Click to see Annotation Detail View

Biological Process
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
response to copper ion  IDA 2307322 RGD 

Objects Annotated

Genes (Rattus norvegicus)
Cyp27b1  (cytochrome P450, family 27, subfamily b, polypeptide 1)

Genes (Mus musculus)
Cyp27b1  (cytochrome P450, family 27, subfamily b, polypeptide 1)

Genes (Homo sapiens)
CYP27B1  (cytochrome P450 family 27 subfamily B member 1)


Additional Information