Ali Khoshnan, PhD

Title(s)Associate Professor of Research Physiology and Neuroscience
SchoolKeck School of Medicine of Usc
Address1501 San Pablo St.
Health Sciences Campus
Los Angeles CA 90033
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    We study the molecular pathogenesis of Huntington’s disease (HD), a genetically inherited neurodegenerative disorder caused by expansion of a polyglutamine repeat in huntingtin protein (HTT). Mutant HTT is amyloidogenic and forms neurotoxic assemblies, which disrupt various signaling pathways and promote neurodegeneration. Patients develop debilitating motor, psychiatric, and cognitive symptoms.

    Areas of interest

    •Use human neurons, brain organoids, mice and Drosophila models to identify pathogenic assemblies of mutant HTT, study the mechanism of how assemblies spread and propagate in the nervous system, and characterize the neuronal pathways, which regulate neurodegeneration in HD.

    •Produce and engineer antibodies and nanobodies targeting the pathogenic conformations of mutant HTT for diagnostic and therapeutic applications.

    •Investigate the impact of microbiota-gut-brain pathways on the pathogenesis of HD in Drosophila and mouse models. In this project, we are characterizing the role of HTT in regulating microbial homeostasis in the gut, examining how mutant HTT disrupts these communications and triggers aberrant expression of genes implicated in brain development and physiology, and identifying the gut-brain circuits, which contribute to the onset of various HD symptoms.

    •Develop gut-based therapies for HD.

    Collapse Bibliographic 
    Collapse Publications
    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Researchers can login to make corrections and additions, or contact us for help. to make corrections and additions.
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    Altmetrics Details PMC Citations indicate the number of times the publication was cited by articles in PubMed Central, and the Altmetric score represents citations in news articles and social media. (Note that publications are often cited in additional ways that are not shown here.) Fields are based on how the National Library of Medicine (NLM) classifies the publication's journal and might not represent the specific topic of the publication. Translation tags are based on the publication type and the MeSH terms NLM assigns to the publication. Some publications (especially newer ones and publications not in PubMed) might not yet be assigned Field or Translation tags.) Click a Field or Translation tag to filter the publications.
    1. Corrigendum: Gut bacteria regulate the pathogenesis of Huntington's disease in Drosophila model. Front Neurosci. 2022; 16:991513. Chongtham A, Yoo JH, Chin TM, Akingbesote ND, Huda A, Marsh JL, Khoshnan A. PMID: 36312028; PMCID: PMC9611772.
      View in: PubMed   Mentions:
    2. Gut Bacteria Regulate the Pathogenesis of Huntington's Disease in Drosophila Model. Front Neurosci. 2022; 16:902205. Chongtham A, Yoo JH, Chin TM, Akingbesote ND, Huda A, Marsh JL, Khoshnan A. PMID: 35757549; PMCID: PMC9215115.
      View in: PubMed   Mentions: 7  
    3. IKKβ signaling mediates metabolic changes in the hypothalamus of a Huntington disease mouse model. iScience. 2022 Feb 18; 25(2):103771. Soylu-Kucharz R, Khoshnan A, Petersén Å. PMID: 35146388; PMCID: PMC8819015.
      View in: PubMed   Mentions: 2  
    4. Amplification of neurotoxic HTTex1 assemblies in human neurons. Neurobiol Dis. 2021 11; 159:105517. Chongtham A, Isas JM, Pandey NK, Rawat A, Yoo JH, Mastro T, Kennedy MB, Langen R, Khoshnan A. PMID: 34563643; PMCID: PMC8943833.
      View in: PubMed   Mentions: 4     Fields:    Translation:HumansAnimalsCells
    5. Small, Seeding-Competent Huntingtin Fibrils Are Prominent Aggregate Species in Brains of zQ175 Huntington's Disease Knock-in Mice. Front Neurosci. 2021; 15:682172. Schindler F, Praedel N, Neuendorf N, Kunz S, Schnoegl S, Mason MA, Taxy BA, Bates GP, Khoshnan A, Priller J, Grimm J, Maier M, Boeddrich A, Wanker EE. PMID: 34239412; PMCID: PMC8257939.
      View in: PubMed   Mentions: 5  
    6. Identification of distinct conformations associated with monomers and fibril assemblies of mutant huntingtin. Hum Mol Genet. 2018 07 01; 27(13):2330-2343. Ko J, Isas JM, Sabbaugh A, Yoo JH, Pandey NK, Chongtham A, Ladinsky M, Wu WL, Rohweder H, Weiss A, Macdonald D, Munoz-Sanjuan I, Langen R, Patterson PH, Khoshnan A. PMID: 29912367; PMCID: PMC6005051.
      View in: PubMed   Mentions: 13     Fields:    Translation:HumansAnimalsCells
    7. IKKβ and mutant huntingtin interactions regulate the expression of IL-34: implications for microglial-mediated neurodegeneration in HD. Hum Mol Genet. 2017 11 01; 26(21):4267-4277. Khoshnan A, Sabbaugh A, Calamini B, Marinero SA, Dunn DE, Yoo JH, Ko J, Lo DC, Patterson PH. PMID: 28973132; PMCID: PMC5886231.
      View in: PubMed   Mentions: 10     Fields:    Translation:HumansAnimalsCells
    8. DJ-1 modulates aggregation and pathogenesis in models of Huntington's disease. Hum Mol Genet. 2014 Feb 01; 23(3):755-66. Sajjad MU, Green EW, Miller-Fleming L, Hands S, Herrera F, Campesan S, Khoshnan A, Outeiro TF, Giorgini F, Wyttenbach A. PMID: 24070869.
      View in: PubMed   Mentions: 22     Fields:    Translation:HumansAnimalsCells
    9. Antibodies and intrabodies against huntingtin: production and screening of monoclonals and single-chain recombinant forms. Methods Mol Biol. 2013; 1010:231-51. Khoshnan A, Ou S, Ko J, Patterson PH. PMID: 23754229.
      View in: PubMed   Mentions: 1     Fields:    Translation:HumansAnimalsCellsPHPublic Health
    10. Elevated IKKα accelerates the differentiation of human neuronal progenitor cells and induces MeCP2-dependent BDNF expression. PLoS One. 2012; 7(7):e41794. Khoshnan A, Patterson PH. PMID: 22848609; PMCID: PMC3407048.
      View in: PubMed   Mentions: 11     Fields:    Translation:HumansAnimalsCells
    11. The role of IκB kinase complex in the neurobiology of Huntington's disease. Neurobiol Dis. 2011 Aug; 43(2):305-11. Khoshnan A, Patterson PH. PMID: 21554955; PMCID: PMC3124142.
      View in: PubMed   Mentions: 23     Fields:    Translation:HumansAnimals
    12. IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome. J Cell Biol. 2009 Dec 28; 187(7):1083-99. Thompson LM, Aiken CT, Kaltenbach LS, Agrawal N, Illes K, Khoshnan A, Martinez-Vincente M, Arrasate M, O'Rourke JG, Khashwji H, Lukacsovich T, Zhu YZ, Lau AL, Massey A, Hayden MR, Zeitlin SO, Finkbeiner S, Green KN, LaFerla FM, Bates G, Huang L, Patterson PH, Lo DC, Cuervo AM, Marsh JL, Steffan JS. PMID: 20026656; PMCID: PMC2806289.
      View in: PubMed   Mentions: 190     Fields:    Translation:HumansAnimalsCells
    13. IKKalpha and IKKbeta regulation of DNA damage-induced cleavage of huntingtin. PLoS One. 2009 Jun 02; 4(6):e5768. Khoshnan A, Ko J, Tescu S, Brundin P, Patterson PH. PMID: 19488402; PMCID: PMC2685016.
      View in: PubMed   Mentions: 21     Fields:    Translation:HumansAnimalsCells
    14. Intrabodies binding the proline-rich domains of mutant huntingtin increase its turnover and reduce neurotoxicity. J Neurosci. 2008 Sep 03; 28(36):9013-20. Southwell AL, Khoshnan A, Dunn DE, Bugg CW, Lo DC, Patterson PH. PMID: 18768695; PMCID: PMC2633448.
      View in: PubMed   Mentions: 57     Fields:    Translation:HumansAnimalsCells
    15. Activation of the IkappaB kinase complex and nuclear factor-kappaB contributes to mutant huntingtin neurotoxicity. J Neurosci. 2004 Sep 15; 24(37):7999-8008. Khoshnan A, Ko J, Watkin EE, Paige LA, Reinhart PH, Patterson PH. PMID: 15371500; PMCID: PMC6729796.
      View in: PubMed   Mentions: 106     Fields:    Translation:HumansAnimalsCells
    16. Antibodies against huntingtin: production and screening of monoclonals and single-chain recombinant forms. Methods Mol Biol. 2004; 277:87-102. Khoshnan A, Ou S, Ko J, Patterson PH. PMID: 15201451.
      View in: PubMed   Mentions:    Fields:    Translation:Humans
    17. Effects of intracellular expression of anti-huntingtin antibodies of various specificities on mutant huntingtin aggregation and toxicity. Proc Natl Acad Sci U S A. 2002 Jan 22; 99(2):1002-7. Khoshnan A, Ko J, Patterson PH. PMID: 11792860; PMCID: PMC117420.
      View in: PubMed   Mentions: 51     Fields:    Translation:HumansCells
    18. The physical association of protein kinase C theta with a lipid raft-associated inhibitor of kappa B factor kinase (IKK) complex plays a role in the activation of the NF-kappa B cascade by TCR and CD28. J Immunol. 2000 Dec 15; 165(12):6933-40. Khoshnan A, Bae D, Tindell CA, Nel AE. PMID: 11120819.
      View in: PubMed   Mentions: 28     Fields:    Translation:HumansCells
    19. Response differences between human CD4(+) and CD8(+) T-cells during CD28 costimulation: implications for immune cell-based therapies and studies related to the expansion of double-positive T-cells during aging. Clin Immunol. 2000 Sep; 96(3):187-97. Laux I, Khoshnan A, Tindell C, Bae D, Zhu X, June CH, Effros RB, Nel A. PMID: 10964536.
      View in: PubMed   Mentions: 29     Fields:    Translation:HumansCells
    20. The NF-kappa B cascade is important in Bcl-xL expression and for the anti-apoptotic effects of the CD28 receptor in primary human CD4+ lymphocytes. J Immunol. 2000 Aug 15; 165(4):1743-54. Khoshnan A, Tindell C, Laux I, Bae D, Bennett B, Nel AE. PMID: 10925251.
      View in: PubMed   Mentions: 65     Fields:    Translation:HumansCells
    21. Primary human CD4+ T cells contain heterogeneous I kappa B kinase complexes: role in activation of the IL-2 promoter. J Immunol. 1999 Nov 15; 163(10):5444-52. Khoshnan A, Kempiak SJ, Bennett BL, Bae D, Xu W, Manning AM, June CH, Nel AE. PMID: 10553070.
      View in: PubMed   Mentions: 6     Fields:    Translation:HumansCells
    22. Hepatitis C virus core protein binds to the cytoplasmic domain of tumor necrosis factor (TNF) receptor 1 and enhances TNF-induced apoptosis. J Virol. 1998 May; 72(5):3691-7. Zhu N, Khoshnan A, Schneider R, Matsumoto M, Dennert G, Ware C, Lai MM. PMID: 9557650; PMCID: PMC109590.
      View in: PubMed   Mentions: 93     Fields:    Translation:HumansAnimalsCells
    23. Involvement of dsRNA virus in the protein composition and growth kinetics of host Trichomonas vaginalis. Arch Virol. 1997; 142(5):939-52. Provenzano D, Khoshnan A, Alderete JF. PMID: 9191859.
      View in: PubMed   Mentions: 27     Fields:    Translation:AnimalsCells
    24. Characterization of double-stranded RNA satellites associated with the Trichomonas vaginalis virus. J Virol. 1995 Nov; 69(11):6892-7. Khoshnan A, Alderete JF. PMID: 7474105; PMCID: PMC189605.
      View in: PubMed   Mentions: 8     Fields:    Translation:AnimalsCells
    25. Unique double-stranded RNAs associated with the Trichomonas vaginalis virus are synthesized by viral RNA-dependent RNA polymerase. J Virol. 1994 Nov; 68(11):7108-14. Khoshnan A, Provenzano D, Alderete JF. PMID: 7933092; PMCID: PMC237149.
      View in: PubMed   Mentions: 8     Fields:    Translation:HumansAnimalsCells
    26. Trichomonas vaginalis with a double-stranded RNA virus has upregulated levels of phenotypically variable immunogen mRNA. J Virol. 1994 Jun; 68(6):4035-8. Khoshnan A, Alderete JF. PMID: 8189538; PMCID: PMC236912.
      View in: PubMed   Mentions: 32     Fields:    Translation:HumansAnimalsCells
    27. Multiple double-stranded RNA segments are associated with virus particles infecting Trichomonas vaginalis. J Virol. 1993 Dec; 67(12):6950-5. Khoshnan A, Alderete JF. PMID: 8230417; PMCID: PMC238153.
      View in: PubMed   Mentions: 18     Fields:    Translation:AnimalsCells
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