Khanna, Richi: search on Z-Library

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1

Subcutaneous administration of recombinant human acid α-glucosidase co-formulated with the pharmacological chaperone AT2220 leads to lysosomal uptake of rhGAA and glycogen reduction in disease-relevant tissues of mice with Pompe disease

Lun, Yi, Pellegrino, Lee, Xu, Su, Feng, Jessie, Soska, Rebecca, Frascella, Michelle, Garcia, Anadina, Flanagan, John J., Lockhart, David J., Valenzano, Kenneth J., Khanna, Richie

Journal:

Molecular Genetics and Metabolism

Year:

2014

Language:

english

File:

PDF, 48 KB

english, 2014

2

Engineering Antibiotic Producers to Overcome the Limitations of Classical Strain Improvement Programs

Lal, Rup, Khanna, Richie, Kaur, Hardeep, Khanna, Monisha, Dhingra, Nidhi, Lal, Sukanya, Gartemann, Karl-Heinz, Eichenlaub, Rudolf, Ghosh, P. K.

Journal:

Critical Reviews in Microbiology

Year:

1996

Language:

english

File:

PDF, 3.99 MB

english, 1996

3

The Pharmacological Chaperone 1-Deoxygalactonojirimycin Reduces Tissue Globotriaosylceramide Levels in a Mouse Model of Fabry Disease

Khanna, Richie, Soska, Rebecca, Lun, Yi, Feng, Jessie, Frascella, Michelle, Young, Brandy, Brignol, Nastry, Pellegrino, Lee, Sitaraman, Sheela A, Desnick, Robert J, Benjamin, Elfrida R, Lockhart, Davi

Journal:

Molecular Therapy

Year:

2010

Language:

english

File:

PDF, 641 KB

english, 2010

4

Structure of acid β-glucosidase with pharmacological chaperone provides insight into Gaucher disease

Lieberman, Raquel L, Wustman, Brandon A, Huertas, Pedro, Powe, Allan C, Pine, Corey W, Khanna, Richie, Schlossmacher, Michael G, Ringe, Dagmar, Petsko, Gregory A

Journal:

Nature Chemical Biology

Year:

2006

Language:

english

File:

PDF, 889 KB

english, 2006

5

Regulation and manipulation of the gene clusters encoding type-I PKSs

Rup Lal, Rekha Kumari, Hardeep Kaur, Richie Khanna, Nidhi Dhingra, Dipika Tuteja

Journal:

Trends in Biotechnology

Year:

2000

Language:

english

File:

PDF, 252 KB

english, 2000

6

Migalastat HCl Reduces Globotriaosylsphingosine (Lyso-Gb3) in Fabry Transgenic Mice and in the Plasma of Fabry Patients

Young-Gqamana, Brandy, Brignol, Nastry, Chang, Hui-Hwa, Khanna, Richie, Soska, Rebecca, Fuller, Maria, Sitaraman, Sheela A., Germain, Dominique P., Giugliani, Roberto, Hughes, Derralynn A., Mehta, Atu

Journal:

Year:

2013

Language:

english

File:

PDF, 951 KB

english, 2013

7

Extrapolation of migalastat tissue concentrations in mice as a predictor of human tissue concentrations

Wu, Shirley, Schmith, Virginia, Khanna, Richie, Johnson, Franklin K.

Journal:

Molecular Genetics and Metabolism

Year:

2020

File:

PDF, 332 KB

2020

8

Co-administration of the pharmacological chaperone AT2221 with a proprietary recombinant human acid alfa-glucosidase leads to greater plasma exposure and substrate reduction compared to alglucosidase alfa

Khanna, Richie, Xu, Su, Hilliard, Deborah, Lun, Yi, Schilling, Adriane, Soska, Rebecca, Nair, Anju, Chang, Kate, Feng, Jessie, Frascella, Michelle, Garcia, Anadina, Pendino, Kimberly, Johnson, Frankli

Journal:

Molecular Genetics and Metabolism

Year:

2016

Language:

english

File:

PDF, 63 KB

english, 2016

9

Activation of transglutaminase in μ-calpain null erythrocytes

Gerald M O’Neill, S.N Prasanna Murthy, Laszlo Lorand, Richie Khanna, Shih-Chun Liu, Manjit Hanspal, Toshihiko Hanada, Athar H Chishti

Journal:

Biochemical and Biophysical Research Communications

Year:

2003

Language:

english

File:

PDF, 232 KB

english, 2003

10

The use of primary myotubes and fibroblasts for the evaluation of pharmacological chaperone therapy in Pompe disease

Westbroek, Wendy, Tamargo, Rafael J., Xiao, Jingbo, Soska, Rebecca, Valenzano, Kenneth J., Goldin, Ehud, Khanna, Richie, Marugan, Juan J., Sidransky, Ellen

Journal:

Molecular Genetics and Metabolism

Year:

2013

Language:

english

File:

PDF, 63 KB

english, 2013

11

Liquid chromotography-tandem mass spectrometry determination of AT2220 in rodent plasma and tissues

Dungan, Leo B., Chang, Hui H., Hamler, Rick, Brignol, Nastry, Xu, Su, Khanna, Richie, Flanagan, John J., Lockhart, David J., Valenzano, Kenneth J., Benjamin, Elfrida R.

Journal:

Molecular Genetics and Metabolism

Year:

2014

Language:

english

File:

PDF, 64 KB

english, 2014

12

Lysosomal dysfunction in gangliosidoses results in secondary accumulation of proteins associated with neurodegeneration

Suraj Saksena, Yi Lun, Serene Keilani, Hadis Williams, Anthony Stevens, Eric Sjoberg, Richie Khanna, Ken Valenzano, David Lockhart, Sam Gandy, Brandon Wustman

Journal:

Molecular Genetics and Metabolism

Year:

2011

Language:

english

File:

PDF, 57 KB

english, 2011

13

Pharmacological chaperones aren't just for mutant enzymes anymore: Co-administration of AT1001 Stabilizes rh∂-Gal A, leading to greater uptake and substrate reduction in fabry patient-derived cells and GLA knockout mice

Richie Khanna, Elfrida R. Benjamin, John J. Flanagan, Lee Pellegrino, Adriane Schilling, Kate Chang, Michelle Frascella, Yi Lun, Mathews Adera, Douglas Greene, David J. Lockhart, Kenneth J. Valenzano

Journal:

Molecular Genetics and Metabolism

Year:

2011

Language:

english

File:

PDF, 61 KB

english, 2011

14

A novel recombinant human acid alpha-glucosidase, ATB200, leads to greater substrate reduction and improvement in Pompe disease-relevant markers compared to alglucosidase alfa in GAA KO mice

Lun, Yi, Xu, Su, Soska, Rebecca, Nair, Anju, Frascella, Michelle, Garcia, Anadina, Gotschall, Russell, Do, Hung, Valenzano, Kenneth J, Khanna, Richie

Journal:

Molecular Genetics and Metabolism

Year:

2017

Language:

english

File:

PDF, 47 KB

english, 2017

15

Poly(A)-binding-protein-mediated regulation of hDcp2 decapping in vitro

Khanna, Richie, Kiledjian, Megerditch

Journal:

The EMBO Journal

Year:

2004

Language:

english

File:

PDF, 212 KB

english, 2004

16

Exploring the use of a co-formulated pharmacological chaperone AT2220 with recombinant human acid alpha-glucosidase for Pompe disease

Khanna, Richie, Xu, Su, Lun, Yi, Soska, Rebecca, Feng, Jessie, Frascella, Michelle, Garcia, Anadina, Flanagan, John, Lockhart, David, Valenzano, Kenneth

Journal:

Molecular Genetics and Metabolism

Year:

2013

Language:

english

File:

PDF, 57 KB

english, 2013

17

The Pharmacological Chaperone AT2220 Increases the Specific Activity and Lysosomal Delivery of Mutant Acid Alpha-Glucosidase, and Promotes Glycogen Reduction in a Transgenic Mouse Model of Pompe Disease

Khanna, Richie, Powe, Allan C., Lun, Yi, Soska, Rebecca, Feng, Jessie, Dhulipala, Rohini, Frascella, Michelle, Garcia, Anadina, Pellegrino, Lee J., Xu, Su, Brignol, Nastry, Toth, Matthew J., Do, Hung

Journal:

Year:

2014

Language:

english

File:

PDF, 8.86 MB

english, 2014

18

The pharmacological chaperone isofagomine increases the activity of the Gaucher disease L444P mutant form of β-glucosidase

Richie Khanna, Elfrida R. Benjamin, Lee Pellegrino, Adriane Schilling, Brigitte A. Rigat, Rebecca Soska, Hadis Nafar, Brian E. Ranes, Jessie Feng, Yi Lun, Allan C. Powe, David J. Palling, Brandon A. W

Journal:

Year:

2010

Language:

english

File:

PDF, 795 KB

english, 2010

19

Glucosylceramide Quantitation in Normal and Glucocerebrosidase-Deficient Mouse Brain and Human Cell Lines

Nastry Brignol, Kate Chang, Rick Hamler, Adriane E. Schilling, Richie Khanna, David J. Lockhart, Sean W. Clark, Elfrida R. Benjamin

Journal:

Molecular Genetics and Metabolism

Year:

2012

Language:

english

File:

PDF, 55 KB

english, 2012

20

Pharmacological Chaperones as Therapeutics for Lysosomal Storage Diseases

Boyd, Robert E., Lee, Gary, Rybczynski, Philip, Benjamin, Elfrida R., Khanna, Richie, Wustman, Brandon A., Valenzano, Kenneth J.

Journal:

Journal of Medicinal Chemistry

Year:

2013

Language:

english

File:

PDF, 1.88 MB

english, 2013

21

Glucosylceramide and glucosylsphingosine quantitation by liquid chromotography-tandem mass spectrometry to enable studies of neuronopathic Gaucher disease

Hamler, Rick, Brignol, Nastry, Morrison, Sean, Hui, Chang H., Dungan, Leo, Boyd, Robert E., Clark, Sean W., Khanna, Richie, Wustman, Brandon W., Flanagan, John F., Valenzano, Kenneth J., Lockhart, Dav

Journal:

Molecular Genetics and Metabolism

Year:

2014

Language:

english

File:

PDF, 46 KB

english, 2014

22

Identification and Characterization of Pharmacological Chaperones to Correct Enzyme Deficiencies in Lysosomal Storage Disorders

Valenzano, Kenneth J., Khanna, Richie, Powe, Allan C., Boyd, Robert, Lee, Gary, Flanagan, John J., Benjamin, Elfrida R.

Journal:

Assay and Drug Development Technologies

Year:

2011

Language:

english

File:

PDF, 1.07 MB

english, 2011

23

Histological examination of the effect of a highly phosphorylated proprietary recombinant human acid alpha-glucosidase on glycogen reduction in disease-relevant muscles of Pompe mice

Lun, Yi, Xu, Su, Gotschall, Russell, Schilling, Adriane, Soska, Rebecca, Frascella, Michelle, Garcia, Anadina, Feng, Jessie, Chang, Kate, Do, Hung, Valenzano, Kenneth J., Khanna, Richie

Journal:

Molecular Genetics and Metabolism

Year:

2015

Language:

english

File:

PDF, 50 KB

english, 2015

24

Improved pharmacological chaperones for the treatment of neuronopathic Gaucher and Parkinson's disease

Sean Clark, Gary Lee, Robert Boyd, Sean Sullivan, Lee Pellegrino, Michelle Frascella, Richie Khanna, Nastry Brignol, Brandon Wustman, Philip Rybczynski, Elfrida Benjamin, Kenneth Valenzano, David J. L

Journal:

Molecular Genetics and Metabolism

Year:

2011

Language:

english

File:

PDF, 56 KB

english, 2011

25

50. The pharmacological chaperone AT1001 and treatment of Fabry disease

Richie Khanna, Elfrida R. Benjamin, Rebecca Soska, Yi Lun, Sheela A. Sitaraman, David J. Palling, David J. Lockhart, Kenneth J. Valenzano

Journal:

Molecular Genetics and Metabolism

Year:

2008

Language:

english

File:

PDF, 42 KB

english, 2008

26

Co-administration With the Pharmacological Chaperone AT1001 Increases Recombinant Human α-Galactosidase A Tissue Uptake and Improves Substrate Reduction in Fabry Mice

Benjamin, Elfrida R, Khanna, Richie, Schilling, Adriane, Flanagan, John J, Pellegrino, Lee J, Brignol, Nastry, Lun, Yi, Guillen, Darlene, Ranes, Brian E, Frascella, Michelle, Soska, Rebecca, Feng, Jes

Journal:

Molecular Therapy

Year:

2012

Language:

english

File:

PDF, 864 KB

english, 2012

27

Novel recombinant human acid α-glucosidase with optimal glycosylation is significantly better than standard of care enzyme replacement for glycogen clearance in skeletal muscles of GAA knock-out mice

Gotschall, Russell, Xu, Su, Lun, Yi, Schilling, Adriane, Soska, Rebecca, Frascella, Michelle, Garcia, Anadina, Feng, Jessie, Chang, Kate, Brignol, Nastry, Khanna, Richie, Valenzano, Kenneth J., Do, Hu

Journal:

Molecular Genetics and Metabolism

Year:

2015

Language:

english

File:

PDF, 54 KB

english, 2015

28

A major advance in the search for more effective therapy for Pompe disease

Raben, Nina, Ralston, Evelyn, Lun, Yi, Ponery, Abdul S., Frascella, Michelle, Garcia, Anadina, Valenzano, Kenneth J., Gotschall, Russel, Do, Hung, Khanna, Richie, Puertollano, Rosa

Journal:

Molecular Genetics and Metabolism

Year:

2018

Language:

english

File:

PDF, 50 KB

english, 2018

29

29. Genetic and pharmacological chaperone modulation of brain GCase activity affects synuclein accumulation in mice

Sean Clark, Ying Sun, You-Hai Xu, Lee Pellegrino, Yi Lun, Michelle Frascella, Richie Khanna, Gregory Grabowski, Brandon Wustman, Ken Valenzano, David Lockhart

Journal:

Molecular Genetics and Metabolism

Year:

2010

Language:

english

File:

PDF, 43 KB

english, 2010

30

Glucosylceramide and Glucosylsphingosine Quantitation by Liquid Chromatography-Tandem Mass Spectrometry to Enable In Vivo Preclinical Studies of Neuronopathic Gaucher Disease

Hamler, Rick L, Brignol, Nastry, Clark, Sean W., Morrison, Sean, Dungan, Leo B., Chang, Hui H., Khanna, Richie, Frascella, Michelle, Valenzano, Kenneth J., Benjamin, Elfrida R., Boyd, Robert E.

Journal:

Analytical Chemistry

Year:

2017

Language:

english

File:

PDF, 1.07 MB

english, 2017

31

Stabilized next-generation recombinant human acid alpha-glucosidase ATB200 clears accumulated glycogen and reverses cellular dysfunction to increase functional muscle strength in a mouse model of Pompe disease

Do, Hung, Lun, Yi, Xu, Su, Soska, Rebecca, Nair, Anju, Frascella, Michelle, Garcia, Anadina, Schilling, Adriane, Valle, Cecilia D, Feng, Jessie, Gotschall, Russell, Valenzano, Kenneth J, Khanna, Richi

Journal:

Molecular Genetics and Metabolism

Year:

2017

Language:

english

File:

PDF, 45 KB

english, 2017

32

55. An adjunct rhGAA ERT-small molecule treatment approach for Pompe disease: Pharmacological chaperone AT2220 prevents rhGAA denaturation and loss of enzyme activity

John J. Flanagan, Richie Khanna, George Neusch, Jessie Feng, Michelle Zdancewicz, Brian Ranes, Rebecca Soska, Terry Yin, Ken J. Valenzano, David J. Lockhart, Hung V. Do

Journal:

Molecular Genetics and Metabolism

Year:

2009

Language:

english

File:

PDF, 43 KB

english, 2009

33

The Importance of Homologous Recombination in the Generation of Large Deletions in Hybrid Plasmids in Amycolatopsis mediterranei

Dipika Tuteja, Meenakshi Dua, Richie Khanna, Nidhi Dhingra, Monisha Khanna, Hardeep Kaur, D.M. Saxena, Rup Lal

Journal:

Year:

2000

Language:

english

File:

PDF, 116 KB

english, 2000

34

Exploring the Use of Pharmacological Chaperone AT3375 Alone and in Combination with Recombinant human ß-Glucosidase for Gaucher Disease

Richie Khanna, Lee Pellegrino, Rebecca Soska, Yi Lun, Jessie Feng, Michelle Frascella, Brian E. Ranes, Darlene Guillen, Anadina Garcia, John J. Flanagan, David J. Lockhart, Sean W. Clark, Kenneth J. V

Journal:

Molecular Genetics and Metabolism

Year:

2012

Language:

english

File:

PDF, 56 KB

english, 2012

35

Development of an Improved Cloning Vector and Transformation System in Amycolatopsis mediterranei (Nocardia mediterranei).

LAL, RUP, KHANNA, RICHIE, DHINGRA, NIDHI, KHANNA, MONISHA, LAL, SUKANAYA

Journal:

The Journal of Antibiotics

Year:

1998

Language:

english

File:

PDF, 1.96 MB

english, 1998

36

Challenges in treating Pompe disease: an industry perspective

Do, Hung V., Khanna, Richie, Gotschall, Russell

Journal:

Annals of translational medicine

Year:

2019

Language:

english

File:

PDF, 1.75 MB

english, 2019

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