Full Text Journal Articles by
Author Gautam Rajpal

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Evaluation of Antisense Oligonucleotides Targeting ATXN3 in SCA3 Mouse Models.

Lauren R Moore, Gautam Rajpal, Ian T Dillingham, Maya Qutob, Kate G Blumenstein, Danielle Gattis, Gene Hung, Holly B Kordasiewicz, Henry L Paulson, Hayley S McLoughlin,

The most common dominantly inherited ataxia, spinocerebellar ataxia type 3 (SCA3), is an incurable neurodegenerative disorder caused by a CAG repeat expansion in the ATXN3 gene that encodes an abnormally long polyglutamine tract in the disease protein, ATXN3. Mice lacking ATXN3 are phenotypically normal; hence, disease gene suppression offers a ... Read more >>

Mol Ther Nucleic Acids (Molecular therapy. Nucleic acids)
[2017, 7:200-210]

Cited: 44 times

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Loss of F-box only protein 2 (Fbxo2) disrupts levels and localization of select NMDA receptor subunits, and promotes aberrant synaptic connectivity.

Graham Atkin, Shannon Moore, Yuan Lu, Rick F Nelson, Nathan Tipper, Gautam Rajpal, Jack Hunt, William Tennant, Johannes W Hell, Geoffrey G Murphy, Henry Paulson,

NMDA receptors (NMDARs) play an essential role in some forms of synaptic plasticity, learning, and memory. Therefore, these receptors are highly regulated with respect to their localization, activation, and abundance both within and on the surface of mammalian neurons. Fundamental questions remain, however, regarding how this complex regulation is achieved. ... Read more >>

J Neurosci (The Journal of neuroscience : the official journal of the Society for Neuroscience)
[2015, 35(15):6165-6178]

Cited: 18 times

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Action of protein disulfide isomerase on proinsulin exit from endoplasmic reticulum of pancreatic β-cells.

Gautam Rajpal, Irmgard Schuiki, Ming Liu, Allen Volchuk, Peter Arvan,

For insulin synthesis, the proinsulin precursor is translated at the endoplasmic reticulum (ER), folds to include its three native disulfide bonds, and is exported to secretory granules for processing and secretion. Protein disulfide isomerase (PDI) has long been assumed to assist proinsulin in this process. Herein we have examined the ... Read more >>

J Biol Chem (The Journal of biological chemistry)
[2012, 287(1):43-47]

Cited: 33 times

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Endoplasmic reticulum oxidoreductin-1-like β (ERO1lβ) regulates susceptibility to endoplasmic reticulum stress and is induced by insulin flux in β-cells.

Cynthia Khoo, Juxiang Yang, Gautam Rajpal, You Wang, Jiangying Liu, Peter Arvan, Doris A Stoffers,

Hyperglycemia increases insulin flux through the endoplasmic reticulum (ER) of pancreatic β-cells, and the unfolded protein response pathway is required to enhance insulin processing. Pancreatic and duodenal homeobox 1 (PDX1), a key pancreatic transcription factor, regulates insulin along with targets involved in insulin processing and secretion. Here we find that ... Read more >>

Endocrinology (Endocrinology)
[2011, 152(7):2599-2608]

Cited: 21 times

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Proinsulin misfolding and diabetes: mutant INS gene-induced diabetes of youth.

Ming Liu, Israel Hodish, Leena Haataja, Roberto Lara-Lemus, Gautam Rajpal, Jordan Wright, Peter Arvan,

Type 1B diabetes (typically with early onset and without islet autoantibodies) has been described in patients bearing small coding sequence mutations in the INS gene. Not all mutations in the INS gene cause the autosomal dominant Mutant INS-gene Induced Diabetes of Youth (MIDY) syndrome, but most missense mutations affecting proinsulin ... Read more >>

Trends Endocrinol Metab (Trends in endocrinology and metabolism: TEM)
[2010, 21(11):652-659]

Cited: 87 times

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Single-chain insulins as receptor agonists.

Gautam Rajpal, Ming Liu, Yi Zhang, Peter Arvan,

Single-chain insulins (SCIs) are single polypeptide chains in which the insulin B-chain links contiguously with the insulin A-chain via an uncleaved connecting peptide. Although direct linkage of insulin B- and A-chains produces SCIs with little insulin receptor binding, biologists have been interested in bioengineering linker peptides that form a flexible ... Read more >>

Mol Endocrinol (Molecular endocrinology (Baltimore, Md.))
[2009, 23(5):679-688]

Cited: 11 times

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Misfolded proinsulin affects bystander proinsulin in neonatal diabetes.

Israel Hodish, Ming Liu, Gautam Rajpal, Dennis Larkin, Ronald W Holz, Aaron Adams, Leanza Liu, Peter Arvan,

It has previously been shown that misfolded mutant Akita proinsulin in the endoplasmic reticulum engages directly in protein complexes either with nonmutant proinsulin or with "hProCpepGFP" (human proinsulin bearing emerald-GFP within the C-peptide), impairing the trafficking of these "bystander" proinsulin molecules (Liu, M., Hodish, I., Rhodes, C. J., and Arvan, ... Read more >>

J Biol Chem (The Journal of biological chemistry)
[2010, 285(1):685-694]

Cited: 42 times

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