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Jenny Libien, MD PhD
Interim Chair, Department of Pathology, Division of Neuropathology
Office location: BSB 4-27
Tel: (718) 270-6304
Fax: (718) 270-3313
Lab: (718) 270-8970
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.
Selected Recent Publications
Libien J and Blaner WS, Retinol and Retinol-Binding Protein in Cerebrospinal Fluid: Can vitamin A take the “Idiopathic” out of Idiopathic Intracranial Hypertension? Journal of Neuro-Ophthalmol. 27: 253-257, 2007.
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.
O'byrne SM, Wongsiriroj N, Libien JM, 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.
Libien J, Sacktor TC, Kass IS. Magnesium blocks the loss of protein kinase C, leads to a transient translocation of PKCa and PKCe, and improves recovery after anoxia in rat hippocampal slices. Brain Res Mol Brain Res.136(1-2):104-111, 2005.
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.
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.
Education and Training
1992 – 2001; MD PhD, SUNY-Downstate
2001-2005; Anatomic Pathology / Neuropathology Residency, New York Presbyterian Hospital - Columbia University Medical Center