CPT: Pharmacometrics & Systems Pharmacology, 2018 Acid sphingomyelinase deficiency (ASMD) is a rare lysosomal storage disorder with heterogeneous clinical manifestations, including hepatosplenomegaly and infiltrative pulmonary disease, and is associated with significant morbidity and mortality ...
Chanchala D. Kaddi +15 more
doaj +1 more source
Interaction of Human Chorionic Gonadotropin (hCG) and Asialo-hCG with Recombinant Human Thyrotropin Receptor. [PDF]
, 1994 hCG is a putative thyroid stimulator. The present studies were undertaken to examine its interaction and that of its desialylated variant asialo-hCG with recombinant human TSH (hTSH) receptor (hTSHr). To this end, we transfected a human thyroid carcinoma
Mann, K. +4 more
core +1 more source
Sphingolipids and cholesterol modulate membrane susceptibility to cytosolic phospholipase A2
Journal of Lipid Research, 2000 Modulation of cytosolic phospholipase A2 (cPLA2) activity by sphingomyelin (SPH), ceramide (Cer), and cholesterol (Chol) was investigated in CHO-2B cells activated by the calcium ionophore A23187 and epinephrine.
Elsa Klapisz +4 more
doaj +1 more source
The essential neutral sphingomyelinase is involved in the trafficking of the variant surface glycoprotein in the bloodstream form of Trypanosoma brucei [PDF]
, 2010 Sphingomyelin is the main sphingolipid in Trypanosoma brucei, the causative agent of African sleeping sickness. In vitro and in vivo characterization of the T.
Smith, Terry K. +2 more
core +1 more source
The FASEB Journal, 2007 Acid sphingomyelinase (ASM) deficiency is an autosomal recessive disorder resulting from a deficiency of the lysosomal enzyme ASM and subsequent accumulation of sphingomyelin (SM). A mouse model of ASM deficiency was created in which accumulation of SM occurs similar to the human form of the disease.
Emily R LaCasse +6 more
openaire +1 more source
Recombinant expression, purification and characterization of human hyaluronidases [PDF]
, 2007 Hyaluronidases and their substrate, hyaluronic acid, are known to play a role in signal transduction events directly correlated with tumor growth and cell migration. Yet correlations with disease progression are controversial due to scarce information on
Hofinger, Edith
core +1 more source
Functional consequences of sphingomyelinase-induced changes in erythrocyte membrane structure [PDF]
, 2012 Contains fulltext : 107961.pdf (Publisher’s version ) (Open Access)Inflammation enhances the secretion of sphingomyelinases (SMases). SMases catalyze the hydrolysis of sphingomyelin into phosphocholine and ceramide.
Dinkla, S. +21 more
core +2 more sources
Acid sphingomyelinase deficiency ameliorates Farber disease
, 2021 Farber disease is a rare lysosomal storage disorder resulting from acid ceramidase deficiency and subsequent ceramide accumulation. No treatments for Farber disease are clinically available, and affected patients have a severely shortened lifespan.
Kleuser, Burkhard (Prof. Dr. rer. nat.) +10 more
core +1 more source
Molecular characterisation of recombinant human pyroglutamyl peptidase (type I) [PDF]
, 2005 Pyroglutamyl Peptidase I (PAP1, EC 3.4.19.3) hydrolytically cleaves pyroglutamic acid (pGlu) from the N-terminal of most pGlu-peptides. In higher organisms Thyrothropin Releasing Hormone is a notable biologically active substrate of PAP1. The sequence of
Vaas, Paul-Roman
core +2 more sources
Acid sphingomyelinase is involved in CEACAM receptor-mediated phagocytosis of Neisseria gonorrhoeae [PDF]
, 2000 The interaction with human phagocytes is a hallmark of symptomatic Neisseria gonorrhoeae infections. Gonococcal outer membrane proteins of the Opa family induce the opsonin-independent uptake of the bacteria that relies on CEACAM receptors and an active ...
K. Ferlinz +22 more
core +2 more sources