Results 31 to 40 of about 1,094 (157)

The role of saposin C in Gaucher disease [PDF]

Molecular Genetics and Metabolism, 2012
Saposin C is one of four homologous proteins derived from sequential cleavage of the saposin precursor protein, prosaposin. It is an essential activator for glucocerebrosidase, the enzyme deficient in Gaucher disease. Gaucher disease is a rare autosomal recessive lysosomal storage disorder caused by mutations in the GBA gene that exhibits vast ...
Rafael J, Tamargo, Arash, Velayati, Ehud, Goldin, Ellen, Sidransky   +3 more
openaire   +2 more sources

A saposin deficiency model in Drosophila: Lysosomal storage, progressive neurodegeneration and sensory physiological decline

Neurobiology of Disease, 2017
Saposin deficiency is a childhood neurodegenerative lysosomal storage disorder (LSD) that can cause premature death within three months of life.
Samantha J. Hindle, Sarita Hebbar, Dominik Schwudke, Christopher J.H. Elliott, Sean T. Sweeney   +4 more
doaj   +1 more source

Temporal changes in prosaposin expression in the rat dentate gyrus after birth. [PDF]

PLoS ONE, 2014
Neurogenesis in the hippocampal dentate gyrus occurs constitutively throughout postnatal life. Adult neurogenesis includes a multistep process that ends with the formation of a postmitotic and functionally integrated new neuron. During adult neurogenesis,
Midori Morishita, Hiroaki Nabeka, Tetsuya Shimokawa, Kyojy Miyawaki, Takuya Doihara, Shouichiro Saito, Naoto Kobayashi, Seiji Matsuda   +7 more
doaj   +1 more source

Saposins utilize two strategies for lipid transfer and CD1 antigen presentation [PDF]

, 2012
Transferring lipid antigens from membranes into CD1 antigen-presenting proteins represents a major molecular hurdle necessary for T-cell recognition. Saposins facilitate this process, but the mechanisms used are not well understood. We found that saposin
Brenner, Michael B.,, Sun, Ying, Grabowski, Gregory A.,, Peng, Wei,, Besra, Gurdyal S.,, Besra, Gurdyal; id_orcid, Besra, Gurdyal S., Minnaard, Adriaan J.,, Barral, DC, Moody, D. Branch, Tatituri, RVV, Tatituri, Raju V. V., Kasmar, Anne,, Bhowruth, Veemal,, Moody, DB, Bhowruth, Veemal, Brenner, Michael B., Peng, Wei, Peng, W, Kasmar, Anne, Grenha, Rosa,, Sun, Ying,, Grenha, R, Moody, D. Branch,, Leon, L, Tatituri, Raju V.V.,, Veerapen, Natacha,, Grabowski, GA, Leon, Luis, Grabowski, Gregory A., Brenner, MB, Minnaard, Adriaan J., Veerapen, Natacha, Barral, Duarte C., C. Barral, Duarte, Minnaard, AJ, Barral, Duarte C.,, León, Luis,, Kasmar, A, Sun, Y, Minnaard, Adriaan J.; id_orcid, Grenha, Rosa   +41 more
core   +1 more source

In silico insights into protein-protein interactions and folding dynamics of the saposin-like domain of Solanum tuberosum aspartic protease. [PDF]

PLoS ONE, 2014
The plant-specific insert is an approximately 100-residue domain found exclusively within the C-terminal lobe of some plant aspartic proteases. Structurally, this domain is a member of the saposin-like protein family, and is involved in plant pathogen ...
Dref C De Moura, Brian C Bryksa, Rickey Y Yada   +2 more
doaj   +1 more source

Temporal gene expression profiling reveals CEBPD as a candidate regulator of brain disease in prosaposin deficient mice

BMC Neuroscience, 2008
Background Prosaposin encodes, in tandem, four small acidic activator proteins (saposins) with specificities for glycosphingolipid (GSL) hydrolases in lysosomes.
Ran Huimin, Zamzow Matt, Williams Michael T, Jia Li, Sun Ying, Quinn Brian, Aronow Bruce J, Vorhees Charles V, Witte David P, Grabowski Gregory A   +9 more
doaj   +1 more source

Accumulation of saposin in dystrophic neurites is linked to impaired lysosomal functions in Alzheimer’s disease brains

Molecular Neurodegeneration, 2021
Neuritic plaques in Alzheimer’s disease (AD) brains refer to β-amyloid (Aβ) plaques surrounded by dystrophic neurites (DNs), activated microglia and reactive astrocytes.
Md Golam Sharoar, Sarah Palko, Yingying Ge, Takaomi C. Saido, Riqiang Yan   +4 more
doaj   +1 more source

Expression of prosaposin and its receptors in the rat cerebellum after kainic acid injection

IBRO Reports, 2017
Prosaposin (PSAP), a highly conserved glycoprotein, is a precursor of saposins A–D. Accumulating evidence suggests that PSAP is a neurotrophic factor that induces differentiation and prevents death in a variety of neuronal cells through the active region
Xuan Li, Hiroaki Nabeka, Shouichiro Saito, Tetsuya Shimokawa, Md. Sakirul Islam Khan, Kimiko Yamamiya, Fengping Shan, Huiling Gao, Cheng Li, Seiji Matsuda   +9 more
doaj   +1 more source

A prosaposin-derived Peptide alleviates kainic Acid-induced brain injury. [PDF]

PLoS ONE, 2015
Four sphingolipid activator proteins (i.e., saposins A-D) are synthesized from a single precursor protein, prosaposin (PS), which exerts exogenous neurotrophic effects in vivo and in vitro.
Hiroaki Nabeka, Tetsuya Shimokawa, Takuya Doihara, Shouichiro Saito, Hiroyuki Wakisaka, Fumihiko Hamada, Naoto Kobayashi, Seiji Matsuda   +7 more
doaj   +1 more source

Structure of PSAP and the IM-1 antibody.

, 2021
An anti-rat PSAP-specific antibody (IM-1) was prepared against the proteolytic portion (the intermediate portion between saposins C and D) of PSAP.
Tetsuya Shimokawa (485032), Miho Taniguchi (11261679), Naoto Kobayashi (485033), Hiroyuki Wakisaka (740748), Takuya Doihara (569215), Seiji Matsuda (485036), Fumihiko Hamada (29532), Farzana Islam (9713464), Hiroaki Nabeka (485031), Kimiko Yamamiya (668075), Md. Sakirul Islam Khan (9713461)   +10 more
core   +1 more source