Ovine neuronal ceroid lipofuscinosis: a large animal model syntenic with the human neuronal ceroid lipofuscinosis variant CLN6.
about
Batten's disease: clues to neuronal protein catabolism in lysosomesProtein product of CLN6 gene responsible for variant late-onset infantile neuronal ceroid lipofuscinosis interacts with CRMP-2Mutations in a novel CLN6-encoded transmembrane protein cause variant neuronal ceroid lipofuscinosis in man and mouseLarge animal models for Batten disease: a reviewA murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human diseaseLysine 43 is trimethylated in subunit C from bovine mitochondrial ATP synthase and in storage bodies associated with batten disease.Glial fibrillary acidic protein is elevated in the lysosomal storage disease classical late-infantile neuronal ceroid lipofuscinosis, but is not a component of the storage material.A missense mutation in canine CLN6 in an Australian shepherd with neuronal ceroid lipofuscinosis.The development and characterisation of complex ovine neuron cultures from fresh and frozen foetal neurons.An enhanced linkage map of the sheep genome comprising more than 1000 lociThe neuronal ceroid-lipofuscinoses.The intracellular location and function of proteins of neuronal ceroid lipofuscinoses.Rapid and Progressive Regional Brain Atrophy in CLN6 Batten Disease Affected Sheep Measured with Longitudinal Magnetic Resonance Imaging.Genetic disorders of sheep in New Zealand: a review and perspective.Location and connectivity determine GABAergic interneuron survival in the brains of South Hampshire sheep with CLN6 neuronal ceroid lipofuscinosisJuvenile neuronal ceroid lipofuscinosis (JNCL) and the eye.Biochemical characterization of a lysosomal protease deficient in classical late infantile neuronal ceroid lipofuscinosis (LINCL) and development of an enzyme-based assay for diagnosis and exclusion of LINCL in human specimens and animal models.Enhanced expression of manganese-dependent superoxide dismutase in human and sheep CLN6 tissuesRemodeling of mitochondrial interior in cardiac lipofuscinosis.Neuronal ceroid-lipofuscinosis in Borderdale sheep.Canine neuronal ceroid lipofuscinoses: Promising models for preclinical testing of therapeutic interventions.
P2860
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P2860
Ovine neuronal ceroid lipofuscinosis: a large animal model syntenic with the human neuronal ceroid lipofuscinosis variant CLN6.
description
1998 nî lūn-bûn
@nan
1998 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած
@hyw
1998 թվականի սեպտեմբերին հրատարակված գիտական հոդված
@hy
1998年の論文
@ja
1998年論文
@yue
1998年論文
@zh-hant
1998年論文
@zh-hk
1998年論文
@zh-mo
1998年論文
@zh-tw
1998年论文
@wuu
name
Ovine neuronal ceroid lipofusc ...... d lipofuscinosis variant CLN6.
@ast
Ovine neuronal ceroid lipofusc ...... d lipofuscinosis variant CLN6.
@en
type
label
Ovine neuronal ceroid lipofusc ...... d lipofuscinosis variant CLN6.
@ast
Ovine neuronal ceroid lipofusc ...... d lipofuscinosis variant CLN6.
@en
prefLabel
Ovine neuronal ceroid lipofusc ...... d lipofuscinosis variant CLN6.
@ast
Ovine neuronal ceroid lipofusc ...... d lipofuscinosis variant CLN6.
@en
P2093
P2860
P356
P1476
Ovine neuronal ceroid lipofusc ...... d lipofuscinosis variant CLN6.
@en
P2093
P2860
P304
P356
10.1136/JMG.35.9.717
P407
P577
1998-09-01T00:00:00Z