The defect in transcellular transport of phosphate in the nephron is located in brush-border membranes in X-linked hypophosphatemia (Hyp mouse model)

Author:

Tenenhouse H. S.,Scriver C. R.

Abstract

We purified renal cortex brush-border membranes from mutant hemizygous hypophosphatemic (Hyp/Y) mice and male control (+/Y) littermates. Tenfold purification of mutant and wild-type membranes was obtained. Phosphate enters +/Y brush-border membrane vesicles by a saturable Na+-dependent arsenate-inhibited component and also by a diffusional component observed in the presence of a potassium gradient. Phosphate is not bound or incorporated significantly by mouse brush-border membrane vesicles. Parallel studies with rat renal cortex brush-border membrane vesicles revealed that phosphate and D-glucose transport in rat and mouse vesicles are similar and have the characteristics reported by other workers. Brush-border membrane vesicles prepared from Hyp/Y renal cortex have significant (p < 0.001) partial loss of phosphate transport on the Na+-dependent arsenate-inhibited component. D-Glucose transport is not affected. Our previous studies reveal that other components of transcellular phosphate flux in kidney are normal. Therefore, we conclude that the mutant gene product in the Hyp mouse is confined to the brush-border membrane. Stability of the X-chromosome in mammalian evolution implies that the same gene product is involved in the classic human disease, familial 'vitamin D resistant' X-linked hypophosphatemia.

Publisher

Canadian Science Publishing

Subject

General Medicine

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