Batten disease and the control of the Fo subunit c pore by cGMP and calcium

European Journal of Paediatric Neurology

Volumn 5, Issue SUPPL. A, 2001, Pages 147-150

  McGeoch, J E ^a   Guidotti, G ^a  

^a HARVARD UNIVERSITY   (United States)

Author keywords

Apoptosis; Batten disease; CGMP gradients; FoATP synthase; Subunit c

Indexed keywords

CALCIUM; CARRIER PROTEIN; CATION; CYCLIC GMP; CYCLIC NUCLEOTIDE; GENE PRODUCT; ION CHANNEL; LIGAND; MUTANT PROTEIN; POLYAMINE; PROTON TRANSPORTING ADENOSINE TRIPHOSPHATE SYNTHASE;

ANIMAL TISSUE; APOPTOSIS; ARTICLE; BIOACCUMULATION; BRAIN; BRAIN GROWTH; CONTROLLED STUDY; ELECTRIC POTENTIAL; ENZYME SUBUNIT; EXCITABILITY; GENE MUTATION; HEART; ION CONDUCTANCE; NERVE DEGENERATION; NEURONAL CEROID LIPOFUSCINOSIS; NONHUMAN; PATHOGENESIS; PRIORITY JOURNAL; REGULATORY MECHANISM; RETINA;

EID: 0034927111     PISSN: 10903798     EISSN: None     Source Type: Journal    
DOI: 10.1053/ejpn.2000.0452     Document Type: Article

References (23)

1. A 0.1-700 Hz current through a voltage-clamped pore: Candidate protein for initiator of neural oscillations
2. Ion pores made of mitochondrial ATP synthase subunit c in the neural plasma membrane and Batten disease
3. 0 subunit c pore
4. Reconsideration of the classification of the neuronal ceroid-lipofuscinoses
5. Neuronal ceroid lipofuscinoses
6. Conversion of neural growth cone responses from repulsion to attraction by cyclic nucleotides
7. 1-ATPase from bovine heart mitochondria
8. 1-ATPase
9. 1-ATPase
10. Molecular architecture of the rotary motor in ATP synthase
11. Cardiac pathology in neuronal ceroid lipofuscinoses: A clinicopathologic correlation in three patients
12. Association of mutations in a lysosomal protein with classic late-infantile neuronal ceroid lipofuscinosis
13. The International Batten Disease Consortium: Isolation of a novel gene underlying Batten disease, CLN3
14. Characterization of the expressed genes for subunit c of mitochondrial ATP synthase in sheep with ceroid lipofuscinosis
15. Different patterns of hydrophobic protein storage in different forms of neuronal ceroid lipofuscinosis (NCL, Batten disease)
16. Apoptosis as the mechanism of neurodegeneration in Batten's disease
17. CLN3 defines a novel anti-apoptotic pathway operative in neurodegeneration and mediated by ceramide
18. Apoptosis in the nervous system
19. Purification and characterization of bovine brain lysosomal pepstatin-insensitive proteinase, the gene product deficient in the human late-infantile neuronal ceroid lipofuscinosis
20. Tripeptidyl peptidase 1, the late infantile neuronal ceroid lipofuscinosis gene product, initiates the lysosomal degradation of subunit c of ATP synthase
21. Calcium binding to the subunit c of E. coli ATP-synthase and possible functional implications in energy coupling
22. A combinatorial approach defines specificities of members of a caspase family and granzyme b. Functional relationships established for key mediators of apoptosis
23. Infantile type of so-called neuronal ceroid-lipofuscinosis