(Q22253405)

CLN6 transmembrane ER protein

1 reference

Palmitoyl-protein thioesterase 1

1 reference

CLN8 transmembrane ER and ERGIC protein

1 reference

tripeptidyl peptidase 1

1 reference

protein catabolic process

1 reference

Journal of Neuroscience Research

G Dawson

1

G

Dawson

1 reference

28 March 2023

S Cho

series ordinal

2

author given names

S

author last names

Cho

1 reference

28 March 2023

language of work or name

English

0 references

publication date

15 April 2000

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published in

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volume

60

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page(s)

133-40

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issue

2

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cites work

Ovine neuronal ceroid lipofuscinosis: a large animal model syntenic with the human neuronal ceroid lipofuscinosis variant CLN6.

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

The subcellular location of the yeast Saccharomyces cerevisiae homologue of the protein defective in the juvenile form of Batten disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

A receptor for the selective uptake and degradation of proteins by lysosomes

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Molecular genetics of palmitoyl-protein thioesterase deficiency in the U.S.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Low molecular weight storage material in infantile ceroid lipofuscinosis (CLN1).

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

A cytoplasmic acyl-protein thioesterase that removes palmitate from G protein alpha subunits and p21(RAS)

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Follow-up study of subunit c of mitochondrial ATP synthase (SCMAS) in Batten disease and in unrelated lysosomal disorders.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

A murine model for juvenile NCL: gene targeting of mouse Cln3

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Palmitoyl-protein thioesterase and the molecular pathogenesis of infantile neuronal ceroid lipofuscinosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Biosynthesis and intracellular targeting of the CLN3 protein defective in Batten disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

A novel assay for lysosomal pepstatin-insensitive proteinase and its application for the diagnosis of late-infantile neuronal ceroid lipofuscinosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Isolation and chromosomal mapping of a mouse homolog of the Batten disease gene CLN3.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Expression of polyglutamine-expanded Huntingtin activates the SEK1-JNK pathway and induces apoptosis in a hippocampal neuronal cell line

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Lipid thioesters derived from acylated proteins accumulate in infantile neuronal ceroid lipofuscinosis: correction of the defect in lymphoblasts by recombinant palmitoyl-protein thioesterase

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

The dynamic role of palmitoylation in signal transduction

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Reversible palmitoylation of signaling proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Spectrum of mutations in the Batten disease gene, CLN3

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

A yeast model for the study of Batten disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

BTN1, a yeast gene corresponding to the human gene responsible for Batten's disease, is not essential for viability, mitochondrial function, or degradation of mitochondrial ATP synthase.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Palmitoylation of endogenous and viral acceptor proteins by fatty acyltransferase (PAT) present in erythrocyte ghosts and in placental membranes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Coding sequence and exon/intron organization of the canine CLN3 (Batten disease) gene and its exclusion as the locus for ceroid-lipofuscinosis in English setter dogs

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Association of mutations in a lysosomal protein with classical late-infantile neuronal ceroid lipofuscinosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Mutational analysis of the defective protease in classic late-infantile neuronal ceroid lipofuscinosis, a neurodegenerative lysosomal storage disorder

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Specific alterations in levels of mannose 6-phosphorylated glycoproteins in different neuronal ceroid lipofuscinoses

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Molecular cloning and expression of palmitoyl-protein thioesterase 2 (PPT2), a homolog of lysosomal palmitoyl-protein thioesterase with a distinct substrate specificity

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Palmitoyl-protein thioesterase, an enzyme implicated in neurodegeneration, is localized in neurons and is developmentally regulated in rat brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

The expression of palmitoyl-protein thioesterase is developmentally regulated in neural tissues but not in nonneural tissues.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Batten disease and mitochondrial pathways of proteolysis

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Apoptosis in the pathogenesis and treatment of disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Lysosomal targeting of palmitoyl-protein thioesterase

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Mutations in the palmitoyl protein thioesterase gene causing infantile neuronal ceroid lipofuscinosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Classical late infantile neuronal ceroid lipofuscinosis fibroblasts are deficient in lysosomal tripeptidyl peptidase I

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

2.2 Mb of contiguous nucleotide sequence from chromosome III of C. elegans

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Studies of atypical JNCL suggest overlapping with other NCL forms.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Palmitoyl-protein thioesterase gene expression in the developing mouse brain and retina: implications for early loss of vision in infantile neuronal ceroid lipofuscinosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

Accumulation of dolichol-linked oligosaccharides in ceroid-lipofuscinosis (Batten disease).

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

7 January 2021

Sphingolipid activator proteins (SAPs) are stored together with glycosphingolipids in the infantile neuronal ceroid-lipofuscinosis (INCL)

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

7 January 2021

Human palmitoyl protein thioesterase: evidence for lysosomal targeting of the enzyme and disturbed cellular routing in infantile neuronal ceroid lipofuscinosis

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

7 January 2021

Signalling functions of protein palmitoylation

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

7 January 2021

The dystrophic retina in multisystem disorders: the electroretinogram in neuronal ceroid lipofuscinoses

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

7 January 2021

Specific delay of degradation of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid lipofuscinosis (Batten disease)

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

7 January 2021

Specific delay in the degradation of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid lipofuscinosis is derived from cellular proteolytic dysfunction rather than structural alteration of subunit c

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

7 January 2021

Enzymatic and molecular biological analysis of palmitoyl protein thioesterase deficiency in infantile neuronal ceroid lipofuscinosis

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

7 January 2021

Biochemical characterization of a palmitoyl acyltransferase activity that palmitoylates myristoylated proteins

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

7 January 2021

Autoacylation of G protein alpha subunits

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

7 January 2021

Increases in Bcl-2 and cleavage of caspase-1 and caspase-3 in human brain after head injury.

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

7 January 2021

The neuronal ceroid-lipofuscinoses

1 reference

https://api.crossref.org/works/10.1002%2F%28SICI%291097-4547%2820000415%2960%3A2%3C133%3A%3AAID-JNR1%3E3.0.CO%3B2-3

7 January 2021