Purification and Characterization of Bovine Brain Lysosomal Pepstatin-Insensitive Proteinase, the Gene Product Deficient in the Human Late-Infantile Neuronal Ceroid Lipofuscinosis

Bovine Brain ◽

Purification And Characterization ◽

Purification and characterization of a GTP-binding protein with a molecular weight of 20,000 in bovine brain membranes. Identification as the rho gene product.

Journal of Biological Chemistry ◽

10.1016/s0021-9258(19)81606-x ◽

1988 ◽

Vol 263 (20) ◽

pp. 9926-9932

Author(s):

K Yamamoto ◽

J Kondo ◽

T Hishida ◽

Y Teranishi ◽

Y Takai

Keyword(s):

Molecular Weight ◽

Gene Product ◽

Binding Protein ◽

Bovine Brain ◽

Purification And Characterization ◽

Gtp Binding Protein ◽

Brain Membranes ◽

Gtp Binding

Distribution and development of CLN2 protein, the late-infantile neuronal ceroid lipofuscinosis gene product

Acta Neuropathologica ◽

10.1007/s004010000321 ◽

2001 ◽

Vol 102 (1) ◽

pp. 20-26 ◽

Cited By ~ 20

Author(s):

Yukiko Kurachi ◽

Akira Oka ◽

Masayuki Itoh ◽

Masashi Mizuguchi ◽

Masaharu Hayashi ◽

...

Keyword(s):

Gene Product ◽

The expression of late infantile neuronal ceroid lipofuscinosis (CLN2) gene product in human brains

Neuroscience Letters ◽

10.1016/s0304-3940(98)00787-3 ◽

1998 ◽

Vol 257 (2) ◽

pp. 113-115 ◽

Cited By ~ 9

Author(s):

Akira Oka ◽

Yukiko Kurachi ◽

Masashi Mizuguchi ◽

Masaharu Hayashi ◽

Sachio Takashima

Keyword(s):

Gene Product ◽

Ceroid Lipofuscinosis ◽

Human Brains

Characterization of Endopeptidase Activity of Tripeptidyl Peptidase-I/CLN2 Protein Which Is Deficient in Classical Late Infantile Neuronal Ceroid Lipofuscinosis

Biochemical and Biophysical Research Communications ◽

10.1006/bbrc.2000.2207 ◽

2000 ◽

Vol 268 (3) ◽

pp. 904-908 ◽

Cited By ~ 50

Author(s):

Junji Ezaki ◽

Mitsue Takeda-Ezaki ◽

Kohei Oda ◽

Eiki Kominami

Keyword(s):

Ceroid Lipofuscinosis ◽

Tripeptidyl Peptidase ◽

Endopeptidase Activity

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

Journal of Neurochemistry ◽

10.1046/j.1471-4159.1999.0730700.x ◽

2002 ◽

Vol 73 (2) ◽

pp. 700-711 ◽

Cited By ~ 63

Author(s):

Istvan Sohar ◽

David E. Sleat ◽

Michel Jadot ◽

Peter Lobel

Keyword(s):

Animal Models ◽

Biochemical Characterization ◽

Lysosomal Protease ◽

Ceroid Lipofuscinosis ◽

Protease Deficient

Production and characterization of recombinant human CLN2 protein for enzyme-replacement therapy in late infantile neuronal ceroid lipofuscinosis

Biochemical Journal ◽

10.1042/0264-6021:3570049 ◽

2001 ◽

Vol 357 (1) ◽

pp. 49 ◽

Cited By ~ 35

Author(s):

Li LIN ◽

Peter LOBEL

Keyword(s):

Enzyme Replacement Therapy ◽

Replacement Therapy ◽

Enzyme Replacement ◽

Characterization of Cln3p, the gene product responsible for juvenile neuronal ceroid lipofuscinosis, as a lysosomal integral membrane glycoprotein

Journal of Neurochemistry ◽

10.1046/j.1471-4159.2003.02132.x ◽

2003 ◽

Vol 87 (5) ◽

pp. 1296-1308 ◽

Cited By ~ 47

Author(s):

Junji Ezaki ◽

Mitsue Takeda-Ezaki ◽

Masato Koike ◽

Yoshiyuki Ohsawa ◽

Hikari Taka ◽

...

Keyword(s):

Gene Product ◽

Membrane Glycoprotein ◽

Juvenile Neuronal Ceroid Lipofuscinosis ◽

Tripeptidyl Peptidase I, the Late Infantile Neuronal Ceroid Lipofuscinosis Gene Product, Initiates the Lysosomal Degradation of Subunit c of ATP Synthase

The Journal of Biochemistry ◽

10.1093/oxfordjournals.jbchem.a022781 ◽

2000 ◽

Vol 128 (3) ◽

pp. 509-516 ◽

Cited By ~ 46

Author(s):

J. Ezaki ◽

M. Takeda-Ezaki ◽

E. Kominami

Keyword(s):

Gene Product ◽

Atp Synthase ◽

Lysosomal Degradation ◽

Subunit C ◽

Ceroid Lipofuscinosis ◽

Tripeptidyl Peptidase

VARIANT LATE INFANTILE NEURONAL CEROID LIPOFUSCINOSIS – A NOVEL C>G MISSENSE MUTATION AT THE CLN8 GENE – THE FIRST CASE OUTSIDE FINLAND OR TURKEY

Neuropediatrics ◽

10.1055/s-2006-943686 ◽

2006 ◽

Vol 37 (S 1) ◽

Author(s):

N Zelnik ◽

M Mahajnah ◽

TC Iancu ◽

M Ziegler

Keyword(s):

Missense Mutation ◽

Ceroid Lipofuscinosis ◽

First Case

A murine model of infantile neuronal ceroid lipofuscinosis - Ultrastructural evaluation of storage in the central nervous system and viscera

Pediatric and Developmental Pathology ◽

10.2350/07-03-0242 ◽

2006 ◽

Vol preprint (2007) ◽

pp. 1

Author(s):

Nancy Galvin ◽

Carole Vogler ◽

Beth Levy ◽

Attila Kovacs ◽

Megan Griffey ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Murine Model ◽

Ceroid Lipofuscinosis ◽

The Central Nervous System