Residual levels of tripeptidyl-peptidase I activity dramatically ameliorate disease in late-infantile neuronal ceroid lipofuscinosis.
about
Interactions of the proteins of neuronal ceroid lipofuscinosis: clues to functionA critical tryptophan and Ca2+ in activation and catalysis of TPPI, the enzyme deficient in classic late-infantile neuronal ceroid lipofuscinosisNovel small molecules potentiate premature termination codon readthrough by aminoglycosidesA tailored mouse model of CLN2 disease: A nonsense mutant for testing personalized therapies.Systemic administration of tripeptidyl peptidase I in a mouse model of late infantile neuronal ceroid lipofuscinosis: effect of glycan modification.Neuropathology of the Mcoln1(-/-) knockout mouse model of mucolipidosis type IV.Large-volume intrathecal enzyme delivery increases survival of a mouse model of late infantile neuronal ceroid lipofuscinosis.Gemfibrozil and fenofibrate, Food and Drug Administration-approved lipid-lowering drugs, up-regulate tripeptidyl-peptidase 1 in brain cells via peroxisome proliferator-activated receptor α: implications for late infantile Batten disease therapy.Therapeutic approaches to the challenge of neuronal ceroid lipofuscinoses.Genetic modulation of apoptotic pathways fails to alter disease course in tripeptidyl-peptidase 1 deficient mice.Chronic Enzyme Replacement to the Brain of a Late Infantile Neuronal Ceroid Lipofuscinosis Mouse Has Differential Effects on Phenotypes of Disease.Proteases as therapeutics.Autosomal recessive spinocerebellar ataxia 7 (SCAR7) is caused by variants in TPP1, the gene involved in classic late-infantile neuronal ceroid lipofuscinosis 2 disease (CLN2 disease).A Basic ApoE-Based Peptide Mediator to Deliver Proteins across the Blood-Brain Barrier: Long-Term Efficacy, Toxicity, and Mechanism.A zebrafish model of CLN2 disease is deficient in tripeptidyl peptidase 1 and displays progressive neurodegeneration accompanied by a reduction in proliferation.Inducible transgenic expression of tripeptidyl peptidase 1 in a mouse model of late-infantile neuronal ceroid lipofuscinosis.Gemfibrozil, food and drug administration-approved lipid-lowering drug, increases longevity in mouse model of late infantile neuronal ceroid lipofuscinosis.
P2860
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P2860
Residual levels of tripeptidyl-peptidase I activity dramatically ameliorate disease in late-infantile neuronal ceroid lipofuscinosis.
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
2008年论文
@zh
2008年论文
@zh-cn
name
Residual levels of tripeptidyl ...... euronal ceroid lipofuscinosis.
@ast
Residual levels of tripeptidyl ...... euronal ceroid lipofuscinosis.
@en
type
label
Residual levels of tripeptidyl ...... euronal ceroid lipofuscinosis.
@ast
Residual levels of tripeptidyl ...... euronal ceroid lipofuscinosis.
@en
prefLabel
Residual levels of tripeptidyl ...... euronal ceroid lipofuscinosis.
@ast
Residual levels of tripeptidyl ...... euronal ceroid lipofuscinosis.
@en
P2093
P2860
P1476
Residual levels of tripeptidyl ...... euronal ceroid lipofuscinosis.
@en
P2093
David E Sleat
Istvan Sohar
Kostantin Dobrenis
Kwi-Hye Kim
Mukarram El-Banna
Peter Lobel
Steven U Walkley
P2860
P304
P356
10.1016/J.YMGME.2008.01.014
P577
2008-03-17T00:00:00Z