Lysosomal accumulation of SCMAS (subunit c of mitochondrial ATP synthase) in neurons of the mouse model of mucopolysaccharidosis III B

Molecular Genetics and Metabolism

Volumn 90, Issue 4, 2007, Pages 393-401

  Ryazantsev, Sergey ^a   Yu, Wei Hong ^a   Zhao, Hui Zhi ^a   Neufeld, Elizabeth F ^a   Ohmi, Kazuhiro ^a  

^a UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

Cryo immuno electron microscopy; Lysosomes; Mouse model; Mucopolysaccharidosis III B; Neuronal pathology; Sanfilippo syndrome type B; Subunit c of mitochondrial ATP synthase

Indexed keywords

GANGLIOSIDE GM3; LYSOSOME ASSOCIATED MEMBRANE PROTEIN 1; LYSOSOME ASSOCIATED MEMBRANE PROTEIN 2; OSMIUM TETRAOXIDE; PROTON TRANSPORTING ADENOSINE TRIPHOSPHATE SYNTHASE;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; CONTROLLED STUDY; ELECTRON MICROSCOPY; ENTORHINAL CORTEX; HISTOPATHOLOGY; LYSOSOME; MOUSE; NERVE CELL; NONHUMAN; PHAGOCYTOSIS; PRIORITY JOURNAL; SANFILIPPO SYNDROME; SOMATOSENSORY CORTEX;

AGING; ANIMALS; CRYOELECTRON MICROSCOPY; DISEASE MODELS, ANIMAL; G(M3) GANGLIOSIDE; GLYCOSAMINOGLYCANS; LYSOSOMAL-ASSOCIATED MEMBRANE PROTEIN 2; LYSOSOME-ASSOCIATED MEMBRANE GLYCOPROTEINS; LYSOSOMES; MICE; MICE, INBRED C57BL; MICE, KNOCKOUT; MITOCHONDRIAL PROTON-TRANSLOCATING ATPASES; MUCOPOLYSACCHARIDOSIS III; PROTEIN SUBUNITS; PYRAMIDAL CELLS; SOMATOSENSORY CORTEX;

EQUUS SUBG. HIPPOTIGRIS; MUS;

EID: 33947575758     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2006.11.006     Document Type: Article

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