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Volume 271, Number 23, Issue of June 7, 1996 pp. 13821-13828
©1996 by The American Society for Biochemistry and Molecular Biology, Inc.

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Coordinate Gene Expression of the 3, 4, and 5 Chains of Collagen Type IV
EVIDENCE FROM A CANINE MODEL OF X-LINKED NEPHRITIS WITH A COL4A5 GENE MUTATION

(Received for publication, December 11, 1995, and in revised form, March 18, 1996)

Paul S. Thorner , Keqin Zheng , Raghuram Kalluri ^¶ , Robert Jacobs and Billy G. Hudson ^¶

From the  Department of Pathology, The Hospital for Sick Children and the University of Toronto, Toronto, Ontario M5G 1X8, Canada, the ^¶ Department of Biochemistry and Molecular Biology, University of Kansas Medical Center, Kansas City, Kansas 66160, and the  Department of Pathology, Ontario Veterinary College and the University of Guelph, Guelph, NIG 2WI, Canada

Canine X-linked hereditary nephritis is an animal model for human X-linked hereditary nephritis with a premature stop codon in the 5(IV) gene of collagen type IV. We used this model to examine the other (IV) chains at the mRNA and protein level in the kidney, since in human X-linked hereditary nephritis, the 3(IV) and 4(IV) chains are often absent from the glomerular basement membrane, although both are encoded by autosomal genes. cDNA probes for the 1(IV)-6(IV) chains were generated from normal dog kidney using the polymerase chain reaction. Sequences were 88% identical at the DNA level and 92% identical at the protein level to the respective human (IV) chains. By Northern analysis, transcripts for the 1(IV), 2(IV), and 6(IV) chains were detected at comparable levels in both normal and affected male dog kidney RNA. As previously shown, the transcript for the 5(IV) chain was reduced to ~10% of normal. Unexpectedly, the 3(IV) and 4(IV) transcripts were both decreased 77% in affected male dog kidney, suggesting a mechanism coordinating the expression of these three basement membrane components. The NC1 domain of collagen type IV isolated from normal dog glomeruli was positive for the 3(IV), 4(IV), and 5(IV) chains by Western blotting. In contrast, in the NC1 domain isolated from affected dog glomeruli, these three chains were not detectable, except for a trace of 3(IV) dimer. In X-linked hereditary nephritis, the absence of the 3(IV) and 4(IV) chains from glomerular basement membrane may reflect factors acting at the transcriptional and/or translational level in addition to the protein assembly level.

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