Human recombinant palmitoyl-protein thioesterase-1 (PPT1) for preclinical evaluation of enzyme replacement therapy for infantile neuronal ceroid lipofuscinosis.
about
Moving towards effective therapeutic strategies for Neuronal Ceroid LipofuscinosisInteractions of the proteins of neuronal ceroid lipofuscinosis: clues to functionNeuroprotection and lifespan extension in Ppt1(-/-) mice by NtBuHA: therapeutic implications for INCLSafety and potential efficacy of gemfibrozil as a supportive treatment for children with late infantile neuronal ceroid lipofuscinosis and other lipid storage disorders.Identification of palmitoyl protein thioesterase 1 in human THP1 monocytes and macrophages and characterization of unique biochemical activities for this enzymePathogenesis and therapies for infantile neuronal ceroid lipofuscinosis (infantile CLN1 disease).Reversible Cysteine Acylation Regulates the Activity of Human Palmitoyl-Protein Thioesterase 1 (PPT1).Intravenous high-dose enzyme replacement therapy with recombinant palmitoyl-protein thioesterase reduces visceral lysosomal storage and modestly prolongs survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis.Histochemical localization of palmitoyl protein thioesterase-1 activityTherapeutic approaches to the challenge of neuronal ceroid lipofuscinoses.Considerations for the treatment of infantile neuronal ceroid lipofuscinosis (infantile Batten disease)Synergistic effects of treating the spinal cord and brain in CLN1 disease.Using Patient-Specific Induced Pluripotent Stem Cells and Wild-Type Mice to Develop a Gene Augmentation-Based Strategy to Treat CLN3-Associated Retinal DegenerationNeural stem cells for disease modeling and evaluation of therapeutics for infantile (CLN1/PPT1) and late infantile (CLN2/TPP1) neuronal ceroid lipofuscinoses.
P2860
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P2860
Human recombinant palmitoyl-protein thioesterase-1 (PPT1) for preclinical evaluation of enzyme replacement therapy for infantile neuronal ceroid lipofuscinosis.
description
2009 nî lūn-bûn
@nan
2009 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2009 թվականի դեկտեմբերին հրատարակված գիտական հոդված
@hy
2009年の論文
@ja
2009年論文
@yue
2009年論文
@zh-hant
2009年論文
@zh-hk
2009年論文
@zh-mo
2009年論文
@zh-tw
2009年论文
@wuu
name
Human recombinant palmitoyl-pr ...... euronal ceroid lipofuscinosis.
@ast
Human recombinant palmitoyl-pr ...... euronal ceroid lipofuscinosis.
@en
type
label
Human recombinant palmitoyl-pr ...... euronal ceroid lipofuscinosis.
@ast
Human recombinant palmitoyl-pr ...... euronal ceroid lipofuscinosis.
@en
prefLabel
Human recombinant palmitoyl-pr ...... euronal ceroid lipofuscinosis.
@ast
Human recombinant palmitoyl-pr ...... euronal ceroid lipofuscinosis.
@en
P2093
P2860
P1476
Human recombinant palmitoyl-pr ...... euronal ceroid lipofuscinosis.
@en
P2093
Jui-Yun Lu
Sandra L Hofmann
P2860
P304
P356
10.1016/J.YMGME.2009.12.002
P577
2009-12-05T00:00:00Z