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  • Type C Niemann-Pick disease: use of hydrophobic amines to study defective cholesterol transport.

Type C Niemann-Pick disease: use of hydrophobic amines to study defective cholesterol transport.

Developmental neuroscience (1991-01-01)
C F Roff, E Goldin, M E Comly, A Cooney, A Brown, M T Vanier, S P Miller, R O Brady, P G Pentchev
ABSTRACT

Niemann-Pick Type C (NPC) disease is a cholesterol lipidosis resulting from defective postlysosomal cholesterol transport. In normal cells this segment of cholesterol trafficking is inhibited by treatment with either U18666A or imipramine. Other compounds are also capable of blocking postlysosomal cholesterol transport: stearylamine, RV-538, and sphinganine inhibit low-density lipoprotein-induced esterification of cholesterol and cause unesterified cholesterol to accumulate in perinuclear vesicles. These vesicles can be stained with filipin to give a staining pattern indistinguishable from that seen in NPC fibroblasts. Because all of these compounds are hydrophobic amines, we conclude that most, if not all, hydrophobic amines block the postlysosomal transport of cholesterol. These results also raise the possibility that an endogenous amine, e.g., sphinganine, may inhibit cholesterol transport in NPC.

MATERIALS
Product Number
Brand
Product Description

Sigma-Aldrich
D-erythro-Dihydrosphingosine, ≥98%
Pricing and availability is not currently available.
Avanti
Sphinganine (d20:0), Avanti Research - A Croda Brand 860674P, powder
Pricing and availability is not currently available.
Avanti
sphinganine-d7, Avanti Research - A Croda Brand 860658P, powder
Pricing and availability is not currently available.
Avanti
sphinganine (d17:0), Avanti Research - A Croda Brand 860654P, powder
Pricing and availability is not currently available.