Submitochondrial distribution and delayed proteolysis of subunit c of the H+-transporting ATP-synthase in ovine ceroid-lipofuscinosis (Q74094899)

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8 November 2019

title

Submitochondrial distribution and delayed proteolysis of subunit c of the H+-transporting ATP-synthase in ovine ceroid-lipofuscinosis (English)

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8 November 2019

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3

Jolly RD

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8 November 2019

author name string

Hughes SM

series ordinal

1

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8 November 2019

Moroni-Rawson P

series ordinal

2

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8 November 2019

Jordan TW

series ordinal

4

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8 November 2019

publication date

1 May 2001

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8 November 2019

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8 November 2019

volume

22

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8 November 2019

issue

9

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8 November 2019

page(s)

1785-1794

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Sequence analysis and mapping of a novel human mitochondrial ATP synthase subunit 9 cDNA (ATP5G3)

8 November 2019

cites work

1 reference

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Mitochondrial ATP synthase subunit c storage in the ceroid-lipofuscinoses (Batten disease)

21 January 2018

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The sequence of the major protein stored in ovine ceroid lipofuscinosis is identical with that of the dicyclohexylcarbodiimide-reactive proteolipid of mitochondrial ATP synthase

21 January 2018

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Characterization of the expressed genes for subunit c of mitochondrial ATP synthase in sheep with ceroid lipofuscinosis

21 January 2018

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Methylated lysine in storage body protein of sheep with hereditary ceroid-lipofuscinosis

21 January 2018

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Use of electrospray ionization mass spectrometry and tandem mass spectrometry to study binding of F0 inhibitors to ceroid lipofuscinosis protein, a model system for subunit c of mitochondrial ATP synthase

21 January 2018

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

21 January 2018

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Tripeptidyl-peptidase I is apparently the CLN2 protein absent in classical late-infantile neuronal ceroid lipofuscinosis

21 January 2018

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

21 January 2018

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A lysosomal proteinase, the late infantile neuronal ceroid lipofuscinosis gene (CLN2) product, is essential for degradation of a hydrophobic protein, the subunit c of ATP synthase.

21 January 2018

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Storage of saposins A and D in infantile neuronal ceroid-lipofuscinosis

21 January 2018

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

21 January 2018

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Batten disease and mitochondrial pathways of proteolysis

21 January 2018

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Variant proteins in ovine ceroid-lipofuscinosis

21 January 2018

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Isolation of subcellular organelles

21 January 2018

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Comparison of the primary structure of nuclear and mitochondrial glutamate dehydrogenase from bovine liver

21 January 2018

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An electron-transport system associated with the outer membrane of liver mitochondria. A biochemical and morphological study

21 January 2018

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The N-acetyl- -D-hexosaminidases of calf and human brain.

21 January 2018

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Enzymatic properties of the inner and outer membranes of rat liver mitochondria

21 January 2018

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Purification of the proton-translocating ATPase from rat liver mitochondria using the detergent 3-[(3-cholamidopropyl)dimethylammonio]-1-propane sulfonate

21 January 2018

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Tricine-sodium dodecyl sulfate-polyacrylamide gel electrophoresis for the separation of proteins in the range from 1 to 100 kDa

21 January 2018

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Batten disease and the ATP synthase subunit c turnover pathway: raising antibodies to subunit c.

21 January 2018

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The effects of peroxisome proliferators on protein abundances in mouse liver

21 January 2018

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Modification of cysteine residues by alkylation. A tool in peptide mapping and protein identification

21 January 2018

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Different patterns of hydrophobic protein storage in different forms of neuronal ceroid lipofuscinosis (NCL, Batten disease)

21 January 2018

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The complete mitochondrial DNA sequence of the domestic sheep (Ovis aries) and comparison with the other major ovine haplotype

21 January 2018

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

21 January 2018

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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

21 January 2018

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Decreased lysosomal subunit c-degrading activity in fibroblasts from patients with late infantile neuronal ceroid lipofuscinosis

21 January 2018

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The ovine model of neuronal ceroid lipofuscinosis (NCL): its contribution to understanding the pathogenesis of Batten disease

21 January 2018

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10.1002/1522-2683(200105)22:9<1785::AID-ELPS1785>3.0.CO;2-L

21 January 2018

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