Alkaline Ceramidase 3 Deficiency Results in Purkinje Cell Degeneration and Cerebellar Ataxia Due to Dyshomeostasis of Sphingolipids in the Brain (Q27310161)

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English	Alkaline Ceramidase 3 Deficiency Results in Purkinje Cell Degeneration and Cerebellar Ataxia Due to Dyshomeostasis of Sphingolipids in the Brain	scientific article

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scholarly article

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Alkaline Ceramidase 3 Deficiency Results in Purkinje Cell Degeneration and Cerebellar Ataxia Due to Dyshomeostasis of Sphingolipids in the Brain (English)

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Alkaline ceramidase 3

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GOA release 2020-03-11

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based on heuristic

inferred from title

cerebellar ataxia

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based on heuristic

inferred from title

Yusuf A. Hannun

18

object named as

Yusuf A Hannun

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author name string

Kai Wang

1

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Ruijuan Xu

2

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Jennifer Schrandt

3

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Prithvi Shah

4

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Yong Z Gong

5

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Chet Preston

6

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Louis Wang

7

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Jae Kyo Yi

8

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Chih-Li Lin

9

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Wei Sun

10

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Demetri D Spyropoulos

11

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Soyoung Rhee

12

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Mingsong Li

13

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Jie Zhou

14

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Shaoyu Ge

15

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Guofeng Zhang

16

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Ashley J Snider

17

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Lina M Obeid

19

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Cungui Mao

20

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language of work or name

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publication date

October 2015

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11

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10

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e1005591

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copyright license

Creative Commons CC0 License

16 October 2015

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April 2022 Public Data File from Crossref

copyright status

copyrighted, dedicated to the public domain by copyright holder

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describes a project that uses

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Europe PubMed Central

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC4608763/fullTextXML

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Ablation of neuronal ceramide synthase 1 in mice decreases ganglioside levels and expression of myelin-associated glycoprotein in oligodendrocytes

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Ceramide synthases at the centre of sphingolipid metabolism and biology

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A deficiency of ceramide biosynthesis causes cerebellar purkinje cell neurodegeneration and lipofuscin accumulation

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Mammalian ceramide synthases

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Substrate specificity, membrane topology, and activity regulation of human alkaline ceramidase 2 (ACER2)

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Alkaline ceramidase 3 (ACER3) hydrolyzes unsaturated long-chain ceramides, and its down-regulation inhibits both cell proliferation and apoptosis

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Ceramidases: regulators of cellular responses mediated by ceramide, sphingosine, and sphingosine-1-phosphate

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The sphingolipid salvage pathway in ceramide metabolism and signaling

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Upregulation of the human alkaline ceramidase 1 and acid ceramidase mediates calcium-induced differentiation of epidermal keratinocytes

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Golgi alkaline ceramidase regulates cell proliferation and survival by controlling levels of sphingosine and S1P

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LASS3 (longevity assurance homologue 3) is a mainly testis-specific (dihydro)ceramide synthase with relatively broad substrate specificity

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Mammalian Lass6 and its related family members regulate synthesis of specific ceramides

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Cloning and characterization of a novel human alkaline ceramidase. A mammalian enzyme that hydrolyzes phytoceramide

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Molecular cloning and characterization of a human mitochondrial ceramidase

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Molecular cloning and characterization of a full-length complementary DNA encoding human acid ceramidase. Identification Of the first molecular lesion causing Farber disease

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Principles of bioactive lipid signalling: lessons from sphingolipids

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Role of ceramide in cellular senescence

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Substrate-specificities of acid and alkaline ceramidases in fibroblasts from patients with Farber disease and controls

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Isolation of cDNA clones encoding human acid sphingomyelinase: occurrence of alternatively processed transcripts

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21 March 2017

Altered cerebellar development in nuclear receptor TAK1/ TR4 null mice is associated with deficits in GLAST(+) glia, alterations in social behavior, motor learning, startle reactivity, and microglia

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21 March 2017

LASS5 is a bona fide dihydroceramide synthase that selectively utilizes palmitoyl-CoA as acyl donor

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Two mammalian longevity assurance gene (LAG1) family members, trh1 and trh4, regulate dihydroceramide synthesis using different fatty acyl-CoA donors

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Identification and characterization of a sphingolipid delta 4-desaturase family

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Cloning and characterization of a mouse endoplasmic reticulum alkaline ceramidase: an enzyme that preferentially regulates metabolism of very long chain ceramides

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Targeted disruption of the even-skipped gene, evx1, causes early postimplantation lethality of the mouse conceptus

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Age-related remodeling of small arteries is accompanied by increased sphingomyelinase activity and accumulation of long-chain ceramides

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Reduced sphingosine kinase-1 and enhanced sphingosine 1-phosphate lyase expression demonstrate deregulated sphingosine 1-phosphate signaling in Alzheimer's disease

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Acid ceramidase promotes nuclear export of PTEN through sphingosine 1-phosphate mediated Akt signaling

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28 September 2017

Radiation-induced acid ceramidase confers prostate cancer resistance and tumor relapse

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Systemic ceramide accumulation leads to severe and varied pathological consequences.

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Increased ceramide content and NFκB signaling may contribute to the attenuation of anabolic signaling after resistance exercise in aged males

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Construction of conditional acid ceramidase knockout mice and in vivo effects on oocyte development and fertility

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Ceramide function in the brain: when a slight tilt is enough.

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Chain length-specific properties of ceramides

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Loss of sphingosine kinase 1/S1P signaling impairs cell growth and survival of neurons and progenitor cells in the developing sensory ganglia

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Brain pathology in Niemann Pick disease type A: insights from the acid sphingomyelinase knockout mice

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Aging of cerebellar Purkinje cells

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The role of the ceramide acyl chain length in neurodegeneration: involvement of ceramide synthases

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Neuronopathic Gaucher disease in the mouse: viable combined selective saposin C deficiency and mutant glucocerebrosidase (V394L) mice with glucosylsphingosine and glucosylceramide accumulation and progressive neurological deficits

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Sphingosine and ceramide signalling in apoptosis.

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Identification of the human sphingolipid C4-hydroxylase, hDES2, and its up-regulation during keratinocyte differentiation

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Involvement of oxidative stress-induced abnormalities in ceramide and cholesterol metabolism in brain aging and Alzheimer's disease

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Ceramidase Deficiency in Farber's Disease (Lipogranulomatosis)

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Bone marrow-derived mesenchymal stem cells prevent the loss of Niemann-Pick type C mouse Purkinje neurons by correcting sphingolipid metabolism and increasing sphingosine-1-phosphate

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Purification and characterization of a membrane-bound nonlysosomal ceramidase from rat brain

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Insertional mutagenesis of the mouse acid ceramidase gene leads to early embryonic lethality in homozygotes and progressive lipid storage disease in heterozygotes

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Mutation in saposin D domain of sphingolipid activator protein gene causes urinary system defects and cerebellar Purkinje cell degeneration with accumulation of hydroxy fatty acid-containing ceramide in mouse

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Programmed cell death induced by ceramide

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10.1371/JOURNAL.PGEN.1005591

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