Results 61 to 70 of about 686,065 (200)

Huntington's disease-associated ankyrin repeat palmitoyl transferases are rate-limiting factors in lysosome formation and fusion.

PLoS Genetics
Protein palmitoylation in the Golgi apparatus is critical for the appropriate sorting of various proteins belonging to secretory and lysosomal systems, and defective palmitoylation can lead to the onset of severe pathologies.
Győző Szenci, Attila Boda, Anikó Nagy, Dorottya Károlyi, András Rubics, Zsombor Szőke, Gergő Falcsik, Tibor Kovács, Péter Lőrincz, Gábor Juhász, Szabolcs Takáts   +10 more
doaj   +1 more source

A European Consortium for Lysosomal Storage Diseases

, 2008
Lysosomes are membrane-enclosed compartments, filled with hydrolytic enzymes that are used for the degradation of macromolecules. Proteins and other substrates are delivered to the lysosomes by various pathways including endocytosis, and autophagy, a ...
ANDRIA, GENEROSO
core  

Effect of collection, transport, processing and storage of blood specimens on the activity of lysosomal enzymes in plasma and leukocytes

Brazilian Journal of Medical and Biological Research, 2000
This study was designed to evaluate the effect of different conditions of collection, transport and storage on the quality of blood samples from normal individuals in terms of the activity of the enzymes ß-glucuronidase, total hexosaminidase ...
M. Burin, C. Dutra-Filho, J. Brum, T. Mauricio, M. Amorim, R. Giugliani   +5 more
doaj   +1 more source

Effect of lantana toxicity on lysosomal and cytosol enzymes in guinea pig liver

, 1983
Oral administration of lantana leaf powder to guinea pigs caused an increase in the hepatic postmitochondrial fraction:homogenate ratios of activities of lysosomal enzymes — acid phosphatase, cathepsin B and DNase II.
Sharma, O.P, Dawra, R.K, Makkar, H.P.S   +2 more
core   +1 more source

Signals involved in protein intracellular sorting [PDF]

, 2005
“…Confusion appears to occur just after the articulation of a major conceptual advance that served to greatly clarify a problem of exceptional importance.”- Ira Mellman, 1996.
Nair, Prashant
core   +1 more source

Severe dilated cardiomyopathy as an unusual finding in a young infant with mucolipidosis type 2 [PDF]

, 2006
A neonate presented with mucopolysaccharidosis-like phenotypic expression and typical signs of dysostosis multiplex but without urinary excretion of glycosaminoglycans.
Mueller, Peter, Moeckel, A., Daehnert, I.   +2 more
core  

Fluorometric Assessment Of Lysosomal Enzymes In Garlic Oil Treated Diabetic Rats

, 2004
The effect of Garlic oil on Lysosomal enzymes in streptozotocin-induced diabetic rats were investigated fluorometrically. The serum lysosomal enzymes assayed include β-glucuronidase, N-acetylglucosaminidase (β-hexosaminidase) (NAG) β-D ...
Adoga, GI, Ohaeri, OC
core   +1 more source

Targeted delivery of glucocerebrosidase to lysosomes: The LYSOTAC (LYSOsome-TArgeting Chimera) technology

Asian Journal of Pharmaceutical Sciences
Lysosomal storage diseases (LSDs) are a group of inherited metabolic disorders caused by misfolding of lysosomal proteins and their degradation via endoplasmic reticulum-associated degradation (ERAD).
Hee-Yeon Kim, Eun Nam Choi, Gee Eun Lee, Sanghwa Yoon, Su Ran Mun, Eui Jung Jung, Minji Kim, Hyomin Lim, Yang Jae Kang, Woo-Jae Park, Yong Tae Kwon, Joo-Won Park   +11 more
doaj   +1 more source

Impaired β-glucocerebrosidase activity and processing in frontotemporal dementia due to progranulin mutations

Acta Neuropathologica Communications, 2019
Loss-of-function mutations in progranulin (GRN) are a major autosomal dominant cause of frontotemporal dementia. Most pathogenic GRN mutations result in progranulin haploinsufficiency, which is thought to cause frontotemporal dementia in GRN mutation ...
Andrew E. Arrant, Jonathan R. Roth, Nicholas R. Boyle, Shreya N. Kashyap, Madelyn Q. Hoffmann, Charles F. Murchison, Eliana Marisa Ramos, Alissa L. Nana, Salvatore Spina, Lea T. Grinberg, Bruce L. Miller, William W. Seeley, Erik D. Roberson   +12 more
doaj   +1 more source

Oncogenic H-Ras up-regulates acid β-hexosaminidase by a mechanism dependent on the autophagy regulator TFEB.

PLoS ONE, 2014
The expression of constitutively active H-RasV12 oncogene has been described to induce proliferative arrest and premature senescence in many cell models.
Lorena Urbanelli, Alessandro Magini, Luisa Ercolani, Krizia Sagini, Alice Polchi, Brunella Tancini, Alessandro Brozzi, Tatiana Armeni, Giovanni Principato, Carla Emiliani   +9 more
doaj   +1 more source