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The function of sphingolipids in the nervous system - Wiley Online
mouse, saposin D knockout mouse, sphingolipid activator protein, sphingolipidosis. ... Sphingolipidoses are inherited metabolic disorders of sphingoglycolipid ...

Sphingolipid and Glycosphingolipid Metabolic Pathways in the Era
Sep 26, 2011 ... Analysis of Sphingolipid Metabolism by “Omic”. Technologies. 6408 .... first by whether they have glucose (GlcCer) or galactose. (GalCer) as the ... biosynthesis has been described as nature's version of combina- torial chemistry. .... be a major contributor to the diseases caused by this family of mycotoxins ...

1-Deoxysphingolipids: mysteries in an enigma 1
May 6, 2015 ... 1A) were first described in a letter to the editors of The ... The latest version is at. JBC Papers in ... also inhibit CerS. Disruption of sphingolipid metabolism by ... sensory and autonomic neuropathy type I disease. (HSAN1) allow ...

Sphingolipid Metabolic Pathway: An Overview of Major Roles
Jul 3, 2013 ... and higher plants, sphinganine thus formed is first hydrox- ylated to phytosphingosine and ... version of ceramide can participate in diametrically opposite ... pathological diseases and sphingolipid metabolism. 3. Sphingolipid ...

L-Serine in disease and development - NCBI
*Department of Pediatric Metabolic Diseases, University Medical Centre Utrecht, KC 03.063.0, P.O. Box 85090, 3508 AB ... Cramer [1] first discovered the amino acid serine in 1865 by ... messenger molecules such as phosphatidylserine and ceramide, .... version by the different isoenzymes plays a role in maintaining.

Sphingolipids: the nexus between Gaucher disease and insulin
The electronic version of this article is the complete one and can be found online at: ... metabolism of sphingolipids are conventionally inborn errors of degradation , ... composition of rafts is altered in cell models of Gaucher disease which may be ... The first of these are the sphingomyelins which have a phosphory- choline or ...

Activities of genes controlling sphingolipid metabolism in human
Activities of genes controlling sphingolipid metabolism in human fibroblasts treated with flavonoids ... First Online: 26 July 2015 ... mucopolysaccharidoses, inherited metabolic diseases with often brain disease symptoms. ...... Academic Edition.

Acid sphingomyelinase is activated in sickle cell - Blood Journal
Running Title: Sphingolipid Metabolism in Sickle Cell Disease. Key Points. 1. Alterations in ... Blood First Edition Paper, prepublished online July 29, 2014; DOI  ...

datasets for sphingolipid metabolism to predict potentially interesting
ery of useful relationships between sphingolipid metabolism and disease, such as the identification of new biomarkers.—-. Momin, A. A., H. Park, ... The online version of this article (available at http://www.jlr.org) con- tains supplementary data ..... datasets from breast cancer cell lines were compared first, then normal breast ...

Advances in Biochemistry & Applications in Medicine - Open Access
sic knowledge of amino acids, carbohydrates, lipids, purines and pyrimidines, and also ... metabolism in 1902 who discovered the first metabolic disorder ...... Underwood's pathology: A clinical approach 6th edition Elsevier Health Sciences .

The GARP complex is required for cellular sphingolipid homeostasis
Inhibition of the first step of de novo sphingolipid synthesis is sufficient to mitigate many ... the sphingolipid metabolic enzyme ceramide synthase, increasing its activity and flux of more ... is underscored by the links of sphingolipid imbalances to disease. ...... 16-bit images were collected using Image iQ (version 1.9; Andor).

Sphingolipid and Ceramide Homeostasis: Potential Therapeutic
1st Simon Alan Young ... A simplified diagram of sphingolipid metabolism in mammals. The key ...... disease are directly and/or indirectly involved in the overac-.

Sphingolipids: metabolism and cell signaling - Department of
Sphingolipids were first described by Johann L.W. Thudichum in A Treatise ... that cause a wide spectrum of diseases of plants and animals (A.H. Merrill, 2001).

Metabolic Syndrome, Neurotoxic 1-Deoxysphingolipids and - PLOS
Jan 23, 2017 ... One third of the CIAP-patients had a metabolic syndrome, significantly less frequent than ... A recent study links DSPN with elevated 1-deoxysphingolipids (1 -deoxySLs) in blood [10] .... Statistical analysis was conducted using GraphPad Prism Version 6. ... In our first control group, 19 patients presented with.

Cell Metabolism - Cell Press
Nov 3, 2016 ... Ceramide effects on adipose metabolism are cell autonomous d ... 14Co-first author ... feature of metabolic disease pathology, as the inability of adipo- ...... version of ceramide into sphingomyelin (i.e., the sphingomyelin.

Ceramides as modulators of cellular and whole-body metabolism
metabolic diseases discussed above, this may be a major mechanism through which ..... ed AMPK phosphorylation by full-length and truncated globular adiponectin .... Ceramide inhibits Akt action by two distinct mechanisms: first, it promotes ...

central nervous system degenerative and metabolic disorders
acquired metabolic disorders, inherited metabolic disorders. ➢ Chapter 5 Genetic ... neurological disease. This is updated to be correct for this year's edition of. Robbins ... discovered first. TOMM40 also .... SPHINGOMYELIN. INHERITED ...

The ABCA7 transporter, brain lipids and Alzheimer's disease
years [10–13]. Shortly after the first early-onset familial ... The link between AD and altered brain lipid metabolism is reviewed here, with particular emphasis on the ... Keywords: ABCA1 ▫ ABCA7 ▫ Alzheimer's disease ▫ amyloid-β ▫ ApoE ▫ ApoJ ▫ brain. ▫ cholesterol ... a nonamyloidogenic version of the peptide [28–30] .

paediatric metabolic conditions of the liver - European Medical Journal
Paediatric metabolic disorders with the most clinical manifestations of deranged hepatic metabolism are discussed. ... present in the first few years of life and have a ..... Sphingolipids are degraded ... 4th edition, Springer Berlin Heidelberg, pp.

Sphingolipids: the nexus between Gaucher disease - Springer Link
Oct 11, 2010 ... Lipids in Health and Disease ... First Online: 11 October 2010 ... The online version of this article (doi:10.1186/1476-511X-9-113) contains supplementary material, which is ..... Inborn Errors of Sphingolipid Metabolism.