Jenny Libien, MD PhD

Office

BSB 4-5

Education and Training

• 1992-2001; MD, PhD, SUNY Downstate
• 2001-2005; Anatomic Pathology / Neuropathology Residency, New York Presbyterian Hospital - Columbia University Medical Center

Research Summary

As a neuropathologist, I am interested in neurologic diseases in general, including memory and movement disorders, demyelinating diseases, autoimmune disorders (especially systemic lupus erythematosus), stroke, central nervous system infections, and brain tumors. Currently, my major research and clinical focus is examining cellular dysfunction and toxicity due to amyloid deposition in Alzheimer's disease and transthyretin amyloidosis.

In collaboration with Dr. Alejandro Ivan Hernandez, we are studying how alterations in PARP1, an enzyme necessary for long-term memory, may contribute to memory impairment in Alzheimer's disease. Changes in PARP1 localization within neurons of the hippocampus appear to be associated with the presence of beta-amyloid plaque and neurofibrillary tangle pathology.

We are studying why different forms of transthyretin (TTR) amyloid deposit in different tissues and signaling pathways by which amyloid deposition leads to tissue damage. TTR amyloidosis (ATTR), a common form of amyloidosis, is a lethal systemic disease caused by a mutation in the TTR gene. Mutations result in a misfolding of protein monomers derived from the tetrameric plasma protein TTR and subsequent fibril formation and accumulation in tissues, leading to cardiomyopathy and/ or polyneuropathy. In the elderly, wild type TTR also can give rise to amyloid, resulting in cardiomyopathy and referred to as Senile Systemic Amyloidosis (SSA). It is not known why or how deposition of the wild-type TTR and different mutations lead to different clinical phenotypes.

Peer Reviewed Journals

• Lee SA, Yang KJZ, Brun PJ, Silvaroli JA, Yuen JJ, Shmarakov I, Jiang H, Feranil JB, Li X, Lackey AI, Krezel W, Leibel RL, Libien J, Storch J, Golczak M, Blaner WS, Retinol-binding protein 2 (RBP2) binds monoacylglycerols and modulates gut endocrine signaling and body weight. Sci Adv. 2020, 6(11). PMID: 32195347
• Regier M, Liang J, Choi A, Verma K, Libien J, Hernandez AI, Evidence for Decreased Nucleolar PARP-1 as an Early Marker of Cognitive Impairment, Neural Plasticity 2019,  PMID: 31827497
• Sapi, E.; Kasliwala, R.S.; Ismail, H.; Torres, J.P.; Oldakowski, M.; Markland, S.; Gaur, G.; Melillo, A.; Eisendle, K.; Liegner, K.B.; Libien, J.; Goldman, J.E. The Long-Term Persistence of Borrelia burgdorferi Antigens and DNA in the Tissues of a Patient with Lyme Disease. Antibiotics 2019, 8, 183. PMID: 31614557
• Bailey DN, George MR, Howell DN, Karcher DS, Libien J, Powell DE, Sanfilippo F. Serving as a Temporary Pathology Chair: "Boon" or "Boondoggle"? Acad Pathol. 2019, 6:2374289519877547. PMID:31598546
• Xia R, Otto C, Zeng J, Momeni A, Kagan J, Kuntz K, Libien J, Achromobacter Endocarditis in Native Cardiac Valves – an Autopsy Case Report and Review of the Literature, Cardiovascular Pathology 2018, 36: 6-10. PMID: 29906520
• Xia R, Otto C, Zeng J, Momeni A, Kagan J, Kuntz K, Libien J, Achromobacter Endocarditis in Native Cardiac Valves – an Autopsy Case Report and Review of the Literature, Cardiovascular Pathology, accepted 1/18/2018 pending revisions.
• Libien J, Kupersmith MJ, Blaner WS, McDermott MP, Gao S, Liu Y, Corbett, Wall M, Role of vitamin A metabolism in IIH: Results from the idiopathic intracranial hypertension treatment trial, J. Neurological Sciences, 371: 78-84, 2017. PMID: 28017254
• Liang J, Libien J, Tanaka Y, Axiotis CA, Shao C, Liu J, Brain pathology case of the month – July 2016- case 2 A 49-year-old man with progressive cranial neuropatholies, Brain Pathol. 26(6): 795-796, 2016. PMID: 27647455
• Li Z, Kabir I, Jiang H, Zhou H, Libien J, Zeng J, Stanek A, Ou P, Li KR, Zhang S, Bui HH, Kuo MS, Park TS, Kim B, Worgall TS, Huan C, Jiang XC, Liver serine palmitoyltransferase activity deficiency in early life impairs adheres junctions and promotes tumorigenesis.  Hepatology.  64(6):2089-2102, 2016.  PMID: 27642075
• Zeng J, Libien J, Shaik F, Wolk J, Hernandez AI. Nucleolar PARP-1 expression is decreased in Alzheimer’s Disease: consequences for epigenetic regulation of rDNA and cognition, Neural Plasticity. 2016. Article ID 8987928
• Mangla S, Choi JH, Barone FC, Novotney C, Libien J, Lin E, Pile-Spellman J. Endovascular external carotid artery occlusion for brain selective targeting: a cerebrovascular swine model, BMC Res Notes. 8:808, 2015. PMID: 26689288.  
• Fà M, Zhang H, Staniszewski A, Saeed F, Schiefer IT, Libien J, Petukhov P, Teich AF, Thatcher GR, Arancio O. Novel selective calpain 1 inhibitors as potential therapeutics in Alzheimer’s disease,  J Alzheimers Dis. 49(3): 707-21, 2015. PMID: 26484927
• Lee DC, Gevorgyan T, Graber HL, Pfeil DS, Xu Y, Mangla S, Barone FC, Libien J, Charchaflieh J, Kral JG, Ramirez SA, Simpson L, Barbour RL, Feasibility of Near Infrared Spectroscopic Tomography for Intraoperative Functional Cerebral Monitoring: A Primate Study, The Journal of Thoracic and Cardiovascular Surgery, J Thorac Cardiovasc Surg. 148(6):3204-10, 2014. PMID: 25439529.   
• Liang J, Libien J, Kunam V, Shao C, Rao C, Ectopic pituitary adenoma associated with an empty sella presenting with hearing loss: case report with literature review, Clinical Neuropathology, 33(3): 197-20,  2014. PMID: 24447694.
• Nirenberg M, Libien J, Vonsattel JP, Fahn S, Multiple System Atrophy in a Patient with the Spinocerebellar Ataxia 3 Gene Mutation. Movement Disorders, 22(2): 251-254, 2007.  PMID: 17133518.
• Ambrosio AL, Williams SC, Lignelli A, Salchow DJ, Spicer G, Libien J, Chin SS, Liebsch NJ, Kazim M, Clinicopathologic Review: Giant Cell Reparative Granuloma of the Orbit.  Neurosurgery. 57(4):773-778, 2005. PMID: 16239891.
• O'byrne SM, Wongsiriroj N, Libien J, Vogel S, Goldberg IJ, Baehr W, Palczewski K, Blaner WS. Retinoid absorption and storage is impaired in mice lacking lecithin: Retinol acyltransferase (LRAT).  J Biol Chem. 280(42): 35647-35657, 2005. PMID: 16115871.
• Libien J, Sacktor TC, Kass IS.  Magnesium blocks the loss of protein kinase C, leads to a transient translocation of PKC and PKC, and improves recovery after anoxia in rat hippocampal slices. Brain Res Mol Brain Res.136(1-2):104-111, 2005. PMID: 15893593.
• McClelland S, Libien JM, Chin SS, Adams DJ, Resor SR Jr, Chan S, Goodman RR. Unusual findings in brain biopsies of two patients with acute magnetic resonance imaging lesions associated with focal seizures.  Epilepsia. 46(9):1495-1501, 2005. PMID: 16146445.
• Mocco J, Laufer I, Mack WJ, Winfree CJ, Libien J, Connolly ES Jr., An extramedullary foramen magnum cavernous malformation presenting with acute subarachnoid hemorrhage: case report and literature review.  Neurosurgery.56(2): E410, 2005. PMID: 15729780.
• Tamargo RJ, Connolly ES Jr, McKhann GM, Khandji A, Chang Y, Libien J, Adams D. Clinicopathological Review: primary angiitis of the central nervous system in association with
  cerebral amyloid angiopathy.  Neurosurgery. 53: 136-143, 2003. PMID: 12823882
• Hernandez AI, Blace N, Crary JF, Serrano PA, Leitges M, Libien JM, Weinstein G, Tcherapanov A, Sacktor TC.  Protein kinase M zeta synthesis from a brain mRNA encoding an independent protein
  kinase C zeta catalytic domain. Implications for the molecular mechanism of memory.  J. Biol. Chem. 278: 40305-40316, 2003.  PMID: 12857744.
• Naik M, Benedikz E, Hernandez I, Libien J, Hrabe J, Valsamus M, Dow-Edwards D, Osman M, Sacktor TC. Expression of Protein Kinase M and the complete protein kinase C isoform family in rat brain. J. Comp. Neurol. 426: 243-358, 2000. PMID: 10982466.

Reviews

• Blaner WS, O’Byrne SM, Wongsiriroj N, Kluwe J, D’Ambrosio D, Jiang H, Schwabe RF, Piantedosi R and Libien J, Hepatic Stellate Cell Lipid Droplets: A Specialized Lipid Droplet for Retinoid Storage, Biochimica et Biophysica Acta, 1791(6): 467-473, 2009. PMID: 19071229.Libien J and Blaner WS, Retinol and Retinol-Binding Protein in Cerebrospinal Fluid:
• Can vitamin A take the “Idiopathic” out of Idiopathic Intracranial Hypertension? J Neuro Ophthalmol, 27(4): 253-257, 2007. PMID: 18090556.

Textbooks and Book Chapters

• Momeni A, Pincus M, Libien J, Introduction to Statistic Methods in Pathology. Springer, Cham Switzerland, 2018.
• Gevorgyan T,  Pfeil DS, Graber HL, Xu Y, Mangla S, Barone FC, Libien J, Charchaflieh J, Barbour RL, Lee DC, Cerebral Monitoring and Surveillance Using High Resolution Functional Optical Imaging, NeuroMethods High Resolution Functional Optical Imaging, Neurovascular Coupling Methods. Springer New York, 2014. 307-330.
• Libien J and Chen WP, Vitamin A in Human Disease, in Vitamins for Prevention of Human Diseases. Wolfgang, Herrmann, Rima Obeid (Eds.), Berlin, New York (DE GRUYTER) 2011.