Abnormal LAMP1 glycosylation may play a role in Niemann-Pick disease, type C pathology

Abnormal LAMP1 glycosylation may play a role in Niemann-Pick disease, type C pathology

Posted by Corey Marcath on

Single Donor Human Cerebrospinal Fluid (CSF) from Innovative Research was used in the following study:

 

Abnormal LAMP1 glycosylation may play a role in Niemann-Pick disease, type C pathology

Niamh X. Cawley ,Caitlin Sojka ,Antony Cougnoux,Anna T. Lyons,Elena‐Raluca Nicoli,Christopher A. Wassif,Forbes D. Porter

PLOS ONE
January 30, 2020

“…A hallmark of Niemann-Pick disease, type C (NPC) is the progressive degeneration of Purkinje neurons in the cerebellum caused by the accumulation of free cholesterol and glycosphingolipids in the lysosome. Recent studies suggest that the state of glycosylation of lysosomal membrane proteins may play a role in disease progression. Our study has identified the presence of a highly glycosylated form of Lysosome Associated Membrane Protein 1 (LAMP1) that correlated spatiotemporally with Purkinje neuron loss… Cerebrospinal fluid from five females and five males of varying age and severity was used in this study. Single donor normal human CSF from five females and five males was purchased from Innovative Research, Novi, MI. All samples were stored at -80˚C prior to being assayed…”

 

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  • Tags: Human Biological Fluids, Single Donor Human Cerebrospinal Fluid (CSF)

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