Characterization of Endopeptidase Activity of Tripeptidyl Peptidase-I/CLN2 Protein Which Is Deficient in Classical Late Infantile Neuronal Ceroid Lipofuscinosis
- 24 February 2000
- journal article
- Published by Elsevier BV in Biochemical and Biophysical Research Communications
- Vol. 268 (3), 904-908
- https://doi.org/10.1006/bbrc.2000.2207
Abstract
No abstract availableKeywords
This publication has 20 references indexed in Scilit:
- Identification of Catalytic Residues of Pepstatin-insensitive Carboxyl Proteinases from Prokaryotes by Site-directed MutagenesisPublished by Elsevier BV ,1999
- Batten's disease: eight genes and still counting?The Lancet, 1999
- Association of Mutations in a Lysosomal Protein with Classical Late-Infantile Neuronal Ceroid LipofuscinosisScience, 1997
- Decreased Lysosomal Subunit c-Degrading Activity in Fibroblasts from Patients with Late Infantile Neuronal Ceroid LipofuscinosisNeuropediatrics, 1997
- 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 cJournal of Neurochemistry, 1996
- Cloning and Expression of an Isovaleryl Pepstatin-Insensitive Carboxyl Proteinase Gene from Xanthomonas sp. T-22The Journal of Biochemistry, 1996
- Specific Delay of Degradation of Mitochondrial ATP Synthase Subunit c in Late Infantile Neuronal Ceroid Lipofuscinosis (Batten Disease)Journal of Neurochemistry, 1995
- [37] Structure, properties, mechanisms, and assays ofcysteine protease inhibitors: Cystatins and E-64 derivativesMethods in Enzymology, 1995
- Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis)Biochemical Journal, 1991
- Batten disease: Past, present, and futureAmerican Journal of Medical Genetics, 1988