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. Author manuscript; available in PMC: 2015 May 13.
Published in final edited form as: Int Rev Neurobiol. 2013;110:165–194. doi: 10.1016/B978-0-12-410502-7.00009-0

Table 8.1.

Neuropathologic features of subtypes of NBIA

Subtype Associated gene Iron deposition Pathologic features
PKAN PANK2 GP, SN NFTs, spheroids
PLAN PLA2G6 GP, SN Lewy bodies, NFTs, spheroids
MPAN c19orf12 GP, SN Lewy bodies, spheroids, tau
ACP CP P, CN, GP, Th, DN GFSB; bizarre astrocytes
NFT FTL P, CN, GP, Th, DN Distorted, iron-laden nuclei
FAHN FA2H GP, SN ???
KRS ATP13A2 CN, P ???
BPAN WDR45 SN, GP Lewy bodies, NFTs, spheroids

GP, globus pallidus; SN, substantia nigra; CN, caudate nucleus; P, putamen; Th, thalamus; DN, dentate nucleus; NFTs, neurofibrillary tangles; GFSB, grumose or foamy spheroid bodies.