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| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
2
|
| pubmed:dateCreated |
1993-12-9
|
| pubmed:abstractText |
The C57BL/6J CPK heterozygous breeders secrete in urine a variant EGF-prohormone with a molecular mass of 154 kDa in addition to the normal 165 kDa EGF-prohormone. The 154 kDa prohormone is secreted as a heterodimer with the normal 165 kDa prohormone. The phenotypically normal littermates, like their parents, secrete the 154 and 165 kDa EGF-prohormones in urine while their cystic siblings secrete neither protein. Examination of renal extracts of normal littermates revealed the presence of the 165 kDa but not the 154 kDa EGF-prohormone; renal extracts of cystic siblings contained neither protein. Cyst fluid, however, contained 56 and 49 kDa EGF-immunoreactive proteins in high concentrations. The data suggest that in the absence of normal 165 kDa prohormone, the 154 kDa EGF-prohormone undergoes proteolysis and that the resultant fragments function as cystogens. Since normal siblings do not acquire renal cystic disease despite expressing the variant 154 kDa EGF-prohormone while the affected littermates, which lack the normal 165 kDa EGF-prohormone, manifest renal cystic disease, we suggest that congenital polycystic kidney disease is due to an inborn defect in the synthesis and secretion of the normal 165 kDa renal EGF-prohormone.
|
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
IM
|
| pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/Epidermal Growth Factor,
http://linkedlifedata.com/resource/pubmed/chemical/Macromolecular Substances,
http://linkedlifedata.com/resource/pubmed/chemical/Protein Precursors,
http://linkedlifedata.com/resource/pubmed/chemical/epidermal growth factor precursor
|
| pubmed:status |
MEDLINE
|
| pubmed:month |
Oct
|
| pubmed:issn |
0006-291X
|
| pubmed:author | |
| pubmed:issnType |
Print
|
| pubmed:day |
29
|
| pubmed:volume |
196
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
892-901
|
| pubmed:dateRevised |
2004-11-17
|
| pubmed:meshHeading |
pubmed-meshheading:8240367-Adult,
pubmed-meshheading:8240367-Animals,
pubmed-meshheading:8240367-Disease Models, Animal,
pubmed-meshheading:8240367-Electrophoresis, Polyacrylamide Gel,
pubmed-meshheading:8240367-Epidermal Growth Factor,
pubmed-meshheading:8240367-Female,
pubmed-meshheading:8240367-Genes, Recessive,
pubmed-meshheading:8240367-Heterozygote,
pubmed-meshheading:8240367-Humans,
pubmed-meshheading:8240367-Macromolecular Substances,
pubmed-meshheading:8240367-Male,
pubmed-meshheading:8240367-Metabolism, Inborn Errors,
pubmed-meshheading:8240367-Mice,
pubmed-meshheading:8240367-Mice, Inbred C57BL,
pubmed-meshheading:8240367-Middle Aged,
pubmed-meshheading:8240367-Molecular Weight,
pubmed-meshheading:8240367-Phenotype,
pubmed-meshheading:8240367-Polycystic Kidney Diseases,
pubmed-meshheading:8240367-Protein Precursors,
pubmed-meshheading:8240367-Reference Values
|
| pubmed:year |
1993
|
| pubmed:articleTitle |
An inborn error in epidermal growth factor prohormone metabolism in a mouse model of autosomal recessive polycystic kidney disease.
|
| pubmed:affiliation |
Department of Pediatrics, Harbor-UCLA Medical Center, Torrance 90509.
|
| pubmed:publicationType |
Journal Article
|