Standard-flow LC and thermal focusing ESI elucidates altered liver proteins in late stage Niemann-Pick, type C1 disease.
Bioanalysis
; 11(11): 1067-1083, 2019 Jun.
Article
em En
| MEDLINE
| ID: mdl-31251104
ABSTRACT
Aim:
Mass spectrometry (MS)-based proteomics, particularly with the development of nano-ESI, have been invaluable to our understanding of altered proteins related to human disease. Niemann-Pick, type C1 (NPC1) disease is a fatal, autosomal recessive, neurodegenerative disorder. The resulting defects include unesterified cholesterol and sphingolipids accumulation in the late endosomal/lysosomal system resulting in organ dysfunction including liver disease. Materials &methods:
First, we performed MS analysis of a complex mammalian proteome using both nano- and standard-flow ESI with the intent of developing a differential proteomics platform using standard-flow ESI. Next, we measured the differential liver proteome in the NPC1 mouse model via label-free quantitative MS using standard-flow ESI.Results:
Using the standard-flow ESI approach, we found altered protein levels including, increased Limp2 and Rab7a in liver tissue of Npc1-/- compared to control mice.Conclusion:
Standard-flow ESI can be a tool for quantitative proteomic studies when sample amount is not limited. Using this method, we have identified new protein markers of NPC1.Palavras-chave
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Base de dados:
MEDLINE
Assunto principal:
Temperatura
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Peptídeos e Proteínas de Sinalização Intracelular
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Doença de Niemann-Pick Tipo C
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Fígado
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Hepatopatias
Idioma:
En
Ano de publicação:
2019
Tipo de documento:
Article