(Q24324788)

GOA release 2020-03-11

1 reference

Alena Pecinová

7

0 references

Pavel Ješina

object named as

Pavel Jesina

6

0 references

Johannes A Mayr

object named as

Johannes A Mayr

1

0 references

author name string

Vendula Havlícková

2

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Franz Zimmermann

3

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Iris Magler

4

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Vilma Kaplanová

5

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Alena Pecinová

7

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Hana Nusková

8

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Johannes Koch

9

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Wolfgang Sperl

10

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Josef Houstek

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

11

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language of work or name

English

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publication date

1 September 2010

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published in

Human Molecular Genetics

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volume

19

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issue

17

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page(s)

3430-9

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cites work

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Subcomplexes of human ATP synthase mark mitochondrial biosynthesis disorders

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Analysis of oxidative phosphorylation complexes in cultured human fibroblasts and amniocytes by blue-native-electrophoresis using mitoplasts isolated with the help of digitonin.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Mitochondrial ATP synthesis in permeabilized cells: assessment of the ATP/O values in situ

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

An evaluation of the measurement of the activities of complexes I-IV in the respiratory chain of human skeletal muscle mitochondria.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Pathogenesis of primary defects in mitochondrial ATP synthesis.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

A mitochondrial DNA microdeletion in a newborn girl with transient lactic acidosis

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Absence of F1-ATPase activity in Kluyveromyces lactis lacking the epsilon subunit

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Severe depletion of mitochondrial DNA in spinal muscular atrophy

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Blue native electrophoresis for isolation of membrane protein complexes in enzymatically active form

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Biochemical and molecular investigations in respiratory chain deficiencies

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Altered properties of mitochondrial ATP-synthase in patients with a T-->G mutation in the ATPase 6 (subunit a) gene at position 8993 of mtDNA

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Assembly of mitochondrial ATP synthase in cultured human cells: implications for mitochondrial diseases

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Mitochondrial diseases and ATPase defects of nuclear origin.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Mitochondrial diseases and genetic defects of ATP synthase.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

TMEM70 protein — A novel ancillary factor of mammalian ATP synthase

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Progress towards understanding disease mechanisms in small vertebrate models of neuronal ceroid lipofuscinosis.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Neuronal ceroid lipofuscinoses

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

GUG is an efficient initiation codon to translate the human mitochondrial ATP6 gene

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Two components in pathogenic mechanism of mitochondrial ATPase deficiency: energy deprivation and ROS production.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Flow-cytometric monitoring of mitochondrial depolarisation: from fluorescence intensities to millivolts

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Deficiency of mitochondrial ATP synthase of nuclear genetic origin

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Temperature-induced states of isolated F1-ATPase affect catalysis, enzyme conformation and high-affinity nucleotide binding sites

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Activities of mitochondrial oxidative phosphorylation enzymes in cultured amniocytes

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Assessment of mitochondrial oxidative phosphorylation in patient muscle biopsies, lymphoblasts, and transmitochondrial cell lines

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Function, structure, and biogenesis of mitochondrial ATP synthase

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Interaction between delta and epsilon subunits of F1-ATPase from pig heart mitochondria. Circular dichroism and intrinsic fluorescence of purified and reconstituted delta epsilon complex

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

The F1Fo-ATPase Complex from Bovine Heart Mitochondria: The Molar Ratio of the Subunits in the Stalk Region Linking the F1and FoDomains

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Sequence and organization of the human mitochondrial genome

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

The structure of the central stalk in bovine F(1)-ATPase at 2.4 A resolution

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

TMEM70 mutations cause isolated ATP synthase deficiency and neonatal mitochondrial encephalocardiomyopathy

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Diminished synthesis of subunit a (ATP6) and altered function of ATP synthase and cytochrome c oxidase due to the mtDNA 2 bp microdeletion of TA at positions 9205 and 9206.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

The epsilon-subunit of ATP synthase from bovine heart mitochondria. Complementary DNA sequence, expression in bovine tissues and evidence of homologous sequences in man and rat

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

The delta- and epsilon-subunits of bovine F1-ATPase interact to form a heterodimeric subcomplex.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Complementation of deletion mutants in the genes encoding the F1-ATPase by expression of the corresponding bovine subunits in yeast S. cerevisiae

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

The expression of subunit c correlates with and thus may limit the biosynthesis of the mitochondrial F0F1-ATPase in brown adipose tissue.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Functional F1-ATPase essential in maintaining growth and membrane potential of human mitochondrial DNA-depleted rho degrees cells

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Factor B and the mitochondrial ATP synthase complex

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Identification of two proteins associated with mammalian ATP synthase

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

The metalloprotease encoded by ATP23 has a dual function in processing and assembly of subunit 6 of mitochondrial ATPase

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Prohibitins interact genetically with Atp23, a novel processing peptidase and chaperone for the F1Fo-ATP synthase.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Biochemical and genetic analysis of 3-methylglutaconic aciduria type IV: a diagnostic strategy

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

A novel deficiency of mitochondrial ATPase of nuclear origin

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Mitochondrial encephalocardio-myopathy with early neonatal onset due to TMEM70 mutation

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Respiratory chain complex V deficiency due to a mutation in the assembly gene ATP12

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

A novel mitochondrial ATP8 gene mutation in a patient with apical hypertrophic cardiomyopathy and neuropathy.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Development of a human mitochondrial oligonucleotide microarray (h-MitoArray) and gene expression analysis of fibroblast cell lines from 13 patients with isolated F1Fo ATP synthase deficiency.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021

Lethal infantile mitochondrial disease with isolated complex I deficiency in fibroblasts but with combined complex I and IV deficiencies in muscle

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ254

7 January 2021