TY - JOUR
T1 - Neuropathologic findings in an aged albino Gorilla
AU - Márquez, M.
AU - Serafin, A.
AU - FernÁndez-Bellon, H.
AU - Serrat, S.
AU - Ferrer-Admetlla, A.
AU - Bertranpetit, J.
AU - Ferrer, I.
AU - Pumarola, M.
N1 - Funding Information:
We thank B. Perez, S. Roca, M. Carmona, and R. Blanco of the Institut de Neuropatologia for their technical assistance; Dr. C. Maté and Dr. J. Fernández-Morán of the Barcelona Zoological Garden for management support, and T. Yohannan for editorial assistance. The authors also wish to acknowledge the care and expert dedication with which the primate curator (M. Teresa Abelló) and primate keepers of the Barcelona Zoo tended the gorilla, particularly during his final years. This work was supported by the Animal Tissue Bank of Catalunya (BTAC), the MCT project SAF2001–4772, PTA-2003–01–00568 and the UAB project PRP2005–09. The genetic project has been funded by grant BOS2003–08070 from the Ministerio de Educación y Ciencia, and by the Direcció General de Recerca, Generalitat de Catalunya (JB). Ultrastructural studies were carried out at the Electron Microscopy Service of the Universitat Autònoma de Barcelona.
Copyright:
Copyright 2009 Elsevier B.V., All rights reserved.
PY - 2008/7
Y1 - 2008/7
N2 - Pallido-nigral spheroids associated with iron deposition have been observed in some aged clinically normal nonhuman primates. In humans, similar findings are observed in neurodegeneration with brain iron accumulation diseases, which, in some cases, show associated mutations in pantothenate kinase 2 gene (PANK2). Here we present an aged gorilla, 40 years old, suffering during the last 2 years of life from progressive tetraparesis, nystagmus, and dyskinesia of the arms, hands, and neck, with accompanying abnormal behavior. The postmortem neuropathologic examination revealed, in addition to aging-associated changes in the brain, numerous corpora amylacea in some brain areas, especially the substantia nigra, and large numbers of axonal spheroids associated with iron accumulation in the internal globus pallidus. Sequencing of the gorilla PANK2 gene failed to detect any mutation. The clinical, neuropathologic, and genetic findings in this gorilla point to an age-related pallido-nigral degeneration that presented PKAN-like neurologic deficits.
AB - Pallido-nigral spheroids associated with iron deposition have been observed in some aged clinically normal nonhuman primates. In humans, similar findings are observed in neurodegeneration with brain iron accumulation diseases, which, in some cases, show associated mutations in pantothenate kinase 2 gene (PANK2). Here we present an aged gorilla, 40 years old, suffering during the last 2 years of life from progressive tetraparesis, nystagmus, and dyskinesia of the arms, hands, and neck, with accompanying abnormal behavior. The postmortem neuropathologic examination revealed, in addition to aging-associated changes in the brain, numerous corpora amylacea in some brain areas, especially the substantia nigra, and large numbers of axonal spheroids associated with iron accumulation in the internal globus pallidus. Sequencing of the gorilla PANK2 gene failed to detect any mutation. The clinical, neuropathologic, and genetic findings in this gorilla point to an age-related pallido-nigral degeneration that presented PKAN-like neurologic deficits.
KW - Axonal dystrophy
KW - Gorilla
KW - Iron
KW - Neurodegeneration with brain iron accumulation
KW - Nonhuman primate
KW - Pallido-nigral degeneration
KW - Pantothenate kinase-associated neurodegeneration
UR - http://www.scopus.com/inward/record.url?scp=54749098470&partnerID=8YFLogxK
U2 - 10.1354/vp.45-4-531
DO - 10.1354/vp.45-4-531
M3 - Artículo
C2 - 18587101
AN - SCOPUS:54749098470
VL - 45
SP - 531
EP - 537
JO - Veterinary Pathology
JF - Veterinary Pathology
SN - 0300-9858
IS - 4
ER -