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Results: 1-25 | 26-28
Results: 1-25/28
NEUROCHEMICAL ALTERATIONS IN THE CEREBELLUM OF A MURINE MODEL OF NIEMANN-PICK TYPE-C DISEASE
Authors: YADID G SOTNIKBARKAI I TORNATORE C BAKERCAIRNS B HARVEYWHITE J PENTCHEV PG GOLDIN E
Citation: G. Yadid et al., NEUROCHEMICAL ALTERATIONS IN THE CEREBELLUM OF A MURINE MODEL OF NIEMANN-PICK TYPE-C DISEASE, Brain research, 799(2), 1998, pp. 250-256
CHOLESTEROL REUTILIZATION DURING MYELINATION OF REGENERATING PNS AXONS IS IMPAIRED IN NIEMANN-PICK DISEASE TYPE-C MICE
Authors: GOODRUM JF PENTCHEV PG
Citation: Jf. Goodrum et Pg. Pentchev, CHOLESTEROL REUTILIZATION DURING MYELINATION OF REGENERATING PNS AXONS IS IMPAIRED IN NIEMANN-PICK DISEASE TYPE-C MICE, Journal of neuroscience research, 49(3), 1997, pp. 389-392
NIEMANN-PICK-C1-DISEASE GENE - HOMOLOGY TO MEDIATORS OF CHOLESTEROL HOMEOSTASIS
Authors: CARSTEA ED MORRIS JA COLEMAN KG LOFTUS SK ZHANG D CUMMINGS C GU J ROSENFELD MA PAVAN WJ KRIZMAN DB NAGLE J POLYMEROPOULOS MH STURLEY SL IOANNOU YA HIGGINS ME COMLY M COONEY A BROWN A KANESKI CR BLANCHETTEMACKIE EJ DWYER NK NEUFELD EB CHANG TY LISCUM L STRAUSS JF OHNO K ZEIGLER M CARMI R SOKOL J MARKIE D ONEILL RR VANDIGGELEN OP ELLEDER M PATTERSON MC BRADY RO VANIER MT PENTCHEV PG TAGLE DA
Citation: Ed. Carstea et al., NIEMANN-PICK-C1-DISEASE GENE - HOMOLOGY TO MEDIATORS OF CHOLESTEROL HOMEOSTASIS, Science, 277(5323), 1997, pp. 228-231
MURINE MODEL OF NIEMANN-PICK-C-DISEASE - MUTATION IN A CHOLESTEROL HOMEOSTASIS GENE
Authors: LOFTUS SK MORRIS JA CARSTEA ED GU JZ CUMMINGS C BROWN A ELLISON J OHNO K ROSENFELD MA TAGLE DA PENTCHEV PG PAVAN WJ
Citation: Sk. Loftus et al., MURINE MODEL OF NIEMANN-PICK-C-DISEASE - MUTATION IN A CHOLESTEROL HOMEOSTASIS GENE, Science, 277(5323), 1997, pp. 232-235
SUBSTANTIAL NARROWING OF THE NIEMANN-PICK-C CANDIDATE INTERVAL BY YEAST ARTIFICIAL CHROMOSOME COMPLEMENTATION
Authors: GU JZ CARSTEA ED CUMMINGS C MORRIS JA LOFTUS SK ZHANG D COLEMAN KG COONEY AM COMLY ME FANDINO L ROFF C TAGLE DA PAVAN WJ PENTCHEV PG ROSENFELD MA
Citation: Jz. Gu et al., SUBSTANTIAL NARROWING OF THE NIEMANN-PICK-C CANDIDATE INTERVAL BY YEAST ARTIFICIAL CHROMOSOME COMPLEMENTATION, Proceedings of the National Academy of Sciences of the United Statesof America, 94(14), 1997, pp. 7378-7383
INTRACELLULAR TRAFFICKING OF THE FREE-CHOLESTEROL DERIVED FROM LDL CHOLESTERYL ESTER IS DEFECTIVE IN-VIVO IN NIEMANN-PICK-C-DISEASE - INSIGHTS ON NORMAL METABOLISM OF HDL AND LDL GAINED FROM THE NP-C MUTATION
Authors: SHAMBUREK RD PENTCHEV PG ZECH LA BLANCHETTEMACKIE J CARSTEA ED VANDENBROEK JM COOPER PS NEUFELD EB PHAIR RD BREWER HB BRADY RO SCHWARTZ CC
Citation: Rd. Shamburek et al., INTRACELLULAR TRAFFICKING OF THE FREE-CHOLESTEROL DERIVED FROM LDL CHOLESTERYL ESTER IS DEFECTIVE IN-VIVO IN NIEMANN-PICK-C-DISEASE - INSIGHTS ON NORMAL METABOLISM OF HDL AND LDL GAINED FROM THE NP-C MUTATION, Journal of lipid research, 38(12), 1997, pp. 2422-2435
IDENTIFICATION AND CHARACTERIZATION OF THE NIEMANN-PICK TYPE-C GENE
Authors: TAGLE DA BROWN K CARSTEA ED MORRIS JA COLEMAN KG LOFTUS SK ZHANG D CUMMINGS C GU J MOSBROOK D ROSENFELD MA PAVAN WJ PENTCHEV PG
Citation: Da. Tagle et al., IDENTIFICATION AND CHARACTERIZATION OF THE NIEMANN-PICK TYPE-C GENE, American journal of human genetics, 61(4), 1997, pp. 283-283
NIEMANN-PICK-C, A MURINE MODEL - MUTATIONS IN NPC1 RESULT IN ABNORMALCHOLESTEROL HOMEOSTASIS AND NEURODEGENERATION
Authors: LOFTUS SK MORRIS JA CARSTEA ED GU JZ CUMMINGS C BROWN A ELLISON J OHNO K ROSENFELD MA PENTCHEV PG TAGLE DA PAVAN WJ
Citation: Sk. Loftus et al., NIEMANN-PICK-C, A MURINE MODEL - MUTATIONS IN NPC1 RESULT IN ABNORMALCHOLESTEROL HOMEOSTASIS AND NEURODEGENERATION, American journal of human genetics, 61(4), 1997, pp. 1496-1496
MOLECULAR-CLONING OF THE MOUSE APOLIPOPROTEIN-D GENE AND ITS UP-REGULATED EXPRESSION IN NIEMANN-PICK DISEASE TYPE-C MOUSE MODEL
Authors: YOSHIDA K CLEAVELAND ES NAGLE JW FRENCH S YASWEN L OHSHIMA T BRADY RO PENTCHEV PG KULKARNI AB
Citation: K. Yoshida et al., MOLECULAR-CLONING OF THE MOUSE APOLIPOPROTEIN-D GENE AND ITS UP-REGULATED EXPRESSION IN NIEMANN-PICK DISEASE TYPE-C MOUSE MODEL, DNA and cell biology, 15(10), 1996, pp. 873-882
NIEMANN-PICK - TYPE-C
Authors: PATTERSON MC PENTCHEV PG
Citation: Mc. Patterson et Pg. Pentchev, NIEMANN-PICK - TYPE-C, Neurology, 46(6), 1996, pp. 1785-1786
INTRACELLULAR TRAFFICKING OF CHOLESTEROL MONITORED WITH A CYCLODEXTRIN
Authors: NEUFELD EB COONEY AM PITHA J DAWIDOWICZ EA DWYER NK PENTCHEV PG BLANCHETTEMACKIE EJ
Citation: Eb. Neufeld et al., INTRACELLULAR TRAFFICKING OF CHOLESTEROL MONITORED WITH A CYCLODEXTRIN, The Journal of biological chemistry, 271(35), 1996, pp. 21604-21613
LYSOSOMAL AND GOLGI MODULATION OF THE INTRACELLULAR-DISTRIBUTION OF CHOLESTEROL IN HUMAN FIBROBLASTS
Authors: DWYER NK COXEY RA PENTCHEV PG BLANCHETTEMACKIE EJ
Citation: Nk. Dwyer et al., LYSOSOMAL AND GOLGI MODULATION OF THE INTRACELLULAR-DISTRIBUTION OF CHOLESTEROL IN HUMAN FIBROBLASTS, Molecular biology of the cell, 6, 1995, pp. 1342-1342
ULTRASTRUCTURAL-CHANGES IN THE LUNG IN NIEMANN-PICK TYPE-C MOUSE
Authors: MANABE T YAMANE T HIGASHI Y PENTCHEV PG SUZUKI K
Citation: T. Manabe et al., ULTRASTRUCTURAL-CHANGES IN THE LUNG IN NIEMANN-PICK TYPE-C MOUSE, Virchows Archiv, 427(1), 1995, pp. 77-83
CULTURED SKIN FIBROBLASTS DERIVED FROM PATIENTS WITH MUCOLIPIDOSIS-4 ARE AUTOFLUORESCENT
Authors: GOLDIN E BLANCHETTEMACKIE EJ DWYER NK PENTCHEV PG BRADY RO
Citation: E. Goldin et al., CULTURED SKIN FIBROBLASTS DERIVED FROM PATIENTS WITH MUCOLIPIDOSIS-4 ARE AUTOFLUORESCENT, Pediatric research, 37(6), 1995, pp. 687-692
CHANGES IN THE LEVELS OF DOLICHOL AND DOLICHYL-PHOSPHATE IN A MURINE MODEL OF NIEMANN-PICKS TYPE-C DISEASE
Authors: SCHEDIN S PENTCHEV PG BRUNK U DALLNER G
Citation: S. Schedin et al., CHANGES IN THE LEVELS OF DOLICHOL AND DOLICHYL-PHOSPHATE IN A MURINE MODEL OF NIEMANN-PICKS TYPE-C DISEASE, Journal of neurochemistry, 65(2), 1995, pp. 670-676
PAIRED-HELICAL-FILAMENT-TAU (PHFTAU) IN NIEMANN-PICK TYPE-C DISEASE IS SIMILAR TO PHFTAU IN ALZHEIMERS-DISEASE
Authors: AUER IA SCHMIDT ML LEE VMY CURRY B SUZUKI K SHIN RW PENTCHEV PG CARSTEA ED TROJANOWSKI JQ
Citation: Ia. Auer et al., PAIRED-HELICAL-FILAMENT-TAU (PHFTAU) IN NIEMANN-PICK TYPE-C DISEASE IS SIMILAR TO PHFTAU IN ALZHEIMERS-DISEASE, Acta Neuropathologica, 90(6), 1995, pp. 547-551
PERIPHERAL-NERVE PATHOLOGY IN NIEMANN-PICK TYPE-C MOUSE
Authors: HIGASHI Y MURAYAMA S PENTCHEV PG SUZUKI K
Citation: Y. Higashi et al., PERIPHERAL-NERVE PATHOLOGY IN NIEMANN-PICK TYPE-C MOUSE, Acta Neuropathologica, 90(2), 1995, pp. 158-163
NEUROFIBRILLARY TANGLES IN NIEMANN-PICK DISEASE TYPE-C
Authors: SUZUKI K PARKER CC PENTCHEV PG KATZ D GHETTI B DAGOSTINO AN CARSTEA ED
Citation: K. Suzuki et al., NEUROFIBRILLARY TANGLES IN NIEMANN-PICK DISEASE TYPE-C, Acta Neuropathologica, 89(3), 1995, pp. 227-238
FREE SPHINGOID BASES IN TISSUES FROM PATIENTS WITH TYPE-C NIEMANN-PICK DISEASE AND OTHER LYSOSOMAL STORAGE DISORDERS
Authors: RODRIGUEZLAFRASSE C ROUSSON R PENTCHEV PG LOUISOT P VANIER MT
Citation: C. Rodriguezlafrasse et al., FREE SPHINGOID BASES IN TISSUES FROM PATIENTS WITH TYPE-C NIEMANN-PICK DISEASE AND OTHER LYSOSOMAL STORAGE DISORDERS, Biochimica et biophysica acta. Molecular basis of disease, 1226(2), 1994, pp. 138-144
THE NIEMANN-PICK C-LESION AND ITS RELATIONSHIP TO THE INTRACELLULAR-DISTRIBUTION AND UTILIZATION OF LDL CHOLESTEROL
Authors: PENTCHEV PG BRADY RO BLANCHETTEMACKIE EJ VANIER MT CARSTEA ED PARKER CC GOLDIN E ROFF CF
Citation: Pg. Pentchev et al., THE NIEMANN-PICK C-LESION AND ITS RELATIONSHIP TO THE INTRACELLULAR-DISTRIBUTION AND UTILIZATION OF LDL CHOLESTEROL, Biochimica et biophysica acta. Molecular basis of disease, 1225(3), 1994, pp. 235-243
NIEMANN-PICK-LIKE LIVER-DISEASE AND REDUCED CHOLESTEROL ESTERIFICATION IN FIBROBLASTS OF 2 MALE INFANTS
Authors: KRISTJANSSON K FINEGOLD MJ PENTCHEV PG BELMONT JW
Citation: K. Kristjansson et al., NIEMANN-PICK-LIKE LIVER-DISEASE AND REDUCED CHOLESTEROL ESTERIFICATION IN FIBROBLASTS OF 2 MALE INFANTS, European journal of pediatrics, 153(5), 1994, pp. 347-351
MUCOLIPIDOSIS-IV FIBROBLASTS SYNTHESIZE NORMAL AMOUNTS OF HYALURONIC-ACID
Authors: GOLDIN E IMAI Y KANESKI CR PENTCHEV PG BRADY RO HASCALL VC
Citation: E. Goldin et al., MUCOLIPIDOSIS-IV FIBROBLASTS SYNTHESIZE NORMAL AMOUNTS OF HYALURONIC-ACID, Journal of inherited metabolic disease, 17(5), 1994, pp. 545-553
DIAGNOSIS OF NIEMANN-PICK DISEASE TYPE-C
Authors: PATTERSON MC PENTCHEV PG
Citation: Mc. Patterson et Pg. Pentchev, DIAGNOSIS OF NIEMANN-PICK DISEASE TYPE-C, The Journal of pediatrics, 124(4), 1994, pp. 655-656
CULTURED ASTROCYTES FROM NIEMANN-PICK, TYPE-C MOUSE-BRAIN HAVE DEFECTIVE TRANSPORT OF APOLIPOPROTEIN-D, DYSFUNCTIONAL LYSOSOMAL PROTON PUMPAND ABERRANT CYSTEINE PROTEINASE(S)
Authors: ASOTRA K SURESH S SHASKAN EG PENTCHEV PG PATEL SC
Citation: K. Asotra et al., CULTURED ASTROCYTES FROM NIEMANN-PICK, TYPE-C MOUSE-BRAIN HAVE DEFECTIVE TRANSPORT OF APOLIPOPROTEIN-D, DYSFUNCTIONAL LYSOSOMAL PROTON PUMPAND ABERRANT CYSTEINE PROTEINASE(S), Journal of neurochemistry, 62, 1994, pp. 190000001-190000001
PRIMARY CULTURED-CELLS AND TRANSFORMED-CELLS FROM NIEMANNPICK, TYPE-CMOUSE LACK A FACTOR ESSENTIAL FOR FIBRIL BUNDLING AND CELL-SHAPE CHANGE IN RESPONSE TO 25-HYDROXYCHOLESTEROL
Authors: PATEL SC SURESH S ROFF CF BLANCHETTEMACKIE EJ ASOTRA K PENTCHEV PG
Citation: Sc. Patel et al., PRIMARY CULTURED-CELLS AND TRANSFORMED-CELLS FROM NIEMANNPICK, TYPE-CMOUSE LACK A FACTOR ESSENTIAL FOR FIBRIL BUNDLING AND CELL-SHAPE CHANGE IN RESPONSE TO 25-HYDROXYCHOLESTEROL, Journal of neurochemistry, 62, 1994, pp. 190000043-190000043
Risultati: 1-25 | 26-28