Deficiencies in sex-regulated expression and levels of two hepatic sterol carrier proteins in a murine model of Niemann-Pick type C disease.
作者： C.F. Roff , A Pastuszyn , J F Strauss , J.T. Billheimer , M.T. Vanier
DOI: 10.1016/S0021-9258(19)49619-1
关键词:
摘要: Hepatic sterol carrier protein-2 (SCP2) and protein-X (SCPx) levels in normal mutant Niemann-Pick Type C mice were determined by immunoblotting with antiserum against rat SCP2. A 14-kDa protein was detected the cytosol fraction a 58-kDa found both cytosolic organellar fractions. Expression of hepatic SCPx developmentally regulated sex-specific pattern. The amounts organelle-associated increased 4-fold during sexual development males but decreased dramatically females. Levels SCP2 much less maturation Adult deficient deficit affected reflected failure to increase maturation. In remained at immature mice. Affected male female also unable maintain level near subnormal During declined animals.
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