[HTML][HTML] Lysosomal dysfunction results in altered energy balance
JC Woloszynek, T Coleman, CF Semenkovich… - Journal of Biological …, 2007 - ASBMB
The mucopolysaccharidosis (MPS) type VII mouse was originally described as the adipose
storage deficiency mouse because of its extreme lean phenotype of unknown etiology. Here,
we show that adipose storage deficiency and lower leptin levels are common to five different
lysosomal storage diseases (LSDs): MPSI, MPSIIIB, MPSVII, Niemann-Pick type A/B, and
infantile neuronal ceroid lipofuscinosis. Elevated circulating pro-inflammatory proteins
(VCAM1 and MCP1) were found in multiple LSDs. Multiple anti-inflammatory strategies …
storage deficiency mouse because of its extreme lean phenotype of unknown etiology. Here,
we show that adipose storage deficiency and lower leptin levels are common to five different
lysosomal storage diseases (LSDs): MPSI, MPSIIIB, MPSVII, Niemann-Pick type A/B, and
infantile neuronal ceroid lipofuscinosis. Elevated circulating pro-inflammatory proteins
(VCAM1 and MCP1) were found in multiple LSDs. Multiple anti-inflammatory strategies …