Primary cultures of renal proximal tubule cells derived from individuals with primary hyperoxaluria.
Urol Res
; 37(3): 127-32, 2009 Jun.
Article
en En
| MEDLINE
| ID: mdl-19283374
ABSTRACT
The primary hyperoxalurias, PH1 and PH2, are inherited disorders caused by deficiencies of alanineglyoxylate aminotransferase and glyoxylate reductase, respectively. Mutations in either of these enzymes leads to endogenous oxalate overproduction primarily in the liver, but most pathological effects are exhibited in the kidney ultimately leading to end-stage renal failure and systemic oxalosis. To provide a non-invasive means of accessing kidney cells from individuals with primary hyperoxaluria, we have derived primary cultures of renal proximal tubule cells from the urine of these patients. The cells stain positively for the epithelial markers pan-cytokeratin and zonula occludens 1 and the proximal tubule marker gamma-glutamyl transpeptidase. Mutation analysis confirmed that the cultured cells had the same genotype as the leucocytes of the patients and also expressed glyoxylate reductase at the mRNA level, illustrating their potential value as a source of renal material from these individuals.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Hiperoxaluria Primaria
/
Túbulos Renales Proximales
Límite:
Adolescent
/
Adult
/
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
Idioma:
En
Revista:
Urol Res
Año:
2009
Tipo del documento:
Article
País de afiliación:
Reino Unido