Protective protein/cathepsin a ppca

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Webb25 sep. 2013 · Proteolytic activation of human cathepsin A. Galactosialidosis is a human lysosomal storage disease caused by deficiency in the multifunctional lysosomal … Webb开馆时间：周一至周日7:00-22:30 周五 7:00-12:00; 我的图书馆

The atomic model of the human protective protein/cathepsin A

http://antibodysystem.com/product/3207.html Webb3 nov. 1995 · Lysosomal protective protein/cathepsin A is a serine carboxypeptidase that forms a complex with β-galactosidase and neuraminidase. The enzyme is synthesized … pub in forster

4MWT: Crystal structure of human PPCA (trigonal crystal form 2)

WebbAlthough cathepsin A has lysosomal protease domains, it also forms a complex with neuraminidase and β-D-galactosidase. Without cathepsin A, the latter two enzymes are … Webb1 jan. 2001 · Human lysosomal N-acetyl-alpha-neuraminidase is deficient in two lysosomal storage disorders, sialidosis, caused by structural mutations in the neuraminidase gene, and galactosialidosis, in which a primary defect of protective protein/cathepsin A (PPCA) leads to a combined deficiency of neuraminidase and beta-D-galactosidase. WebbCathepsin A, also known as Protective Protein Cathepsin A (PPCA), is a serine carboxypeptidase that is required for correct lysosomal transportation. Cathepsin A is synthesized as a 54 kD zymogen and is processed into an enzymatically active two-chain form in lysosomes. pub in gairloch

Intermittent enzyme replacement therapy with recombinant …

Recombinant Anti-Protective protein/Cathepsin A (PPCA) antibody ...

WebbCTSA, Lysosomal protective protein, Protective protein cathepsin A, Cathepsin A, Carboxypeptidase L, Protective protein for beta-galactosidase, PPGB, Carboxypeptidase C, PPCA. Species. Human . Shipping. In general, proteins are provided as lyophilized powder/frozen liquid. They are shipped ... WebbProtective Protein/Cathepsin A (PPCA) Deficiency. CTSA Deficiency. B-Galactosidase. Testing Algorithm. Delineates situations when tests are added to the initial order. This includes reflex and additional tests. See Lysosomal Storage Disorders Diagnostic Algorithm, Part 1. ... hotel hendricks how many roomsWebbCathepsin A is a multifunctional enzyme that expresses deaminidase and esterase activities at neutral pH and carboxypeptidase activity at acidic pH. Also known as protective protein, its association with beta -galactosidase ( beta -gal) and neuraminidase is essential for beta -gal stability and neuraminidase activation in the lysosomes. pub in freeland mi

"Webb25 sep. 2013 · Proteolytic activation of human cathepsin A. Galactosialidosis is a human lysosomal storage disease caused by deficiency in the multifunctional lysosomal … " - Protective protein/cathepsin a ppca

Protective protein/cathepsin a ppca

Webb2 aug. 2013 · This defines the enzyme protective function. On the other hand, studies of the physiological role of PPCA/CTSA as a serine carboxypeptidase/deamidase/esterase demonstrated a role of the enzyme in the inactivation of selected neuropeptides, like substance P, oxytocin and endothelin I [ 7 ].

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Webb22 nov. 2024 · We found that this receptor-ligand interaction recruits the eukaryotic sialidase, NEU1, together with its chaperone protein, protective protein/cathepsin A (PPCA), to surface-expressed MUC1 21, 22. Further, we found that NEU1 desialylates the MUC1-ED to unmask cryptic binding sites for flagellin-expressing Pa 21, 23 (Fig. 1). WebbEnter the email address you signed up with and we'll email you a reset link.

Webb1 mars 2005 · GLB1 forms a complex with protective protein cathepsin A (PPCA), alpha neuraminidase (NEU1), and galactosamine 6‐sulphate sulfatase (GALNS) inside lysosomes, while EBP binds to PPCA and NEU1 on the cell surface. We investigated the function of the GLB1 and EBP… View on Wiley doi.org Save to Library Create Alert Cite 52 … WebbLysosomal protective protein/ cathepsin A is a serine carboxypeptidase that forms a complex with beta-galactosidase and neuraminidase [22]. Analytical, diagnostic and therapeutic context of CTSA The deleterious effect of such mutations was confirmed by the complete absence of the PPCA protein on Western blots [18].

Webb16 apr. 2002 · Protective protein appears to be essential for both the activity of beta-galactosidase and neuraminidase, it associates with these enzymes and exerts a … Webb20 jan. 1998 · Human protective protein/cathepsin A (PPCA), a serine carboxypeptidase, forms a multienzyme complex with β-galactosidase and neuraminidase and is required for the intralysosomal activity and stability of these two glycosidases.

Webb20 jan. 1998 · Human protective protein/cathepsin A (PPCA), a serine carboxypeptidase, forms a multienzyme complex with β-galactosidase and neuraminidase and is required …

Webb20 jan. 1998 · Human protective protein/cathepsin A (PPCA), a serine carboxypeptidase, forms a multienzyme complex with beta-galactosidase and neuraminidase and is … pub in funtingtonWebb26 maj 2024 · Within the lysosomes, Neu1 forms a complex with protective protein cathepsin A (PPCA), which protects Neu1 activity (Pshezhetsky and Ashmarina, 2001). Since Neu1 activity was found in the culture medium of BV-2 microglia, we tested whether cathepsin activity was also present in this conditioned medium. pub in fremantleWebbProduct name Recombinant Human Protective protein/Cathepsin A (PPCA) See all Protective protein/Cathepsin A (PPCA) proteins and peptides Purity > 95 % SDS-PAGE. … pub in gestione a romaWebbDie Ursache für die Galaktosialidose ist eine stark verminderte Aktivität der Enzyme α-D- Neuraminidase und β-Galactosidase, die durch einen Defekt des bi-funktionalen Proteins Protective Protein/Cathepsin A (PPCA) („Schutzprotein“) verursacht wird. Zusammen mit der Neuraminidase und der β-Galaktosidase bildet das PPCA einen ... hotel hendaye gareWebb26 sep. 2024 · lysosomal protective protein, carboxypeptidase C, carboxypeptidase L, protective protein cathepsin A, protective protein for beta-galactosidase. GeneRIFs: Gene References Into Functions. ... Results describe the hydrodynamic properties of PPCA, NEU1, and a complex of the two proteins and identified multiple binding sites on both ... hotel henri iv rive gauche tripadvisorWebbRecombinant Anti-Protective protein/Cathepsin A (PPCA) antibody [EPR10434] (ab181129) Datasheet. SDS. Certificate of Compliance. Submit a review Submit a question … pub in gosforthWebbThe glycosylated precursor proteins were taken up via the mannose 6-phosphate receptors, and the cathepsin A, alpha-neuraminidase and beta-galactosidase (beta-Gal) activities … hotel hembacher hof rednitzhembach 91126