Table 1.

Summary of individuals studied and distribution of 1C2 and calcium channel aggregates

ParticipantsCAG repeat-unitAreas studiedDistributions of aggregates
SCA6SCA1SCA2SCA3TBP1C2A6RPT-C
1C2 is the antipolyglutamine antibody, and A6RPT-C is the anticalcium channel antibody.
ND = not determined; FCx = the frontal cortex; TCx = the temporal cortex; MSA = multiple system atrophy; MJD = Machado–Joseph disease; HD = Huntington’s disease; DRPLA = dentatorubral-pallidoluysian atrophy; SCA = spinocerebellar ataxia; TBP = TATA-binding protein.
SCA6
Patient 111 /2227 /2822 /2214 /2735 /35FCx, TCx, hippocampus, striatum, midbrain, pons, medulla, cerebellum, spinal cordPurkinje cell cytoplasm ≫ nucleusPurkinje cell cytoplasm only
Patient 213 /2227 /2722 /2214 /2035 /35FCx, TCx, hippocampus, striatum, midbrain, pons, medulla, cerebellum, spinal cordPurkinje cell cytoplasm ≫ nucleusPurkinje cell cytoplasm only
Patient 313 /2228 /2922 /2219 /2935 /35FCx, TCx, hippocampus, striatum, midbrain, pons, medulla, cerebellum, spinal cordPurkinje cell cytoplasm ≫ nucleusPurkinje cell cytoplasm only
Patient 415 /22NDNDNDNDPons, medulla, cerebellumPurkinje cell cytoplasm ≫ nucleusPurkinje cell cytoplasm only
Patient 513 /24NDNDNDNDCerebellumPurkinje cell cytoplasm ≫ nucleusPurkinje cell cytoplasm only
Patient 616 /20NDNDNDNDCerebellumPurkinje cell cytoplasm ≫ nucleusPurkinje cell cytoplasm only
Controls
Normal controls, n = 3FCx, TCx, hippocampus, striatum, midbrain, pons, medulla, cerebellum, spinal cordNot presentNot present
AD, n = 2CerebellumNot presentNot present
MSA, n = 3CerebellumNot presentNot present
MJD/SCA3, n = 3Pons, cerebellumPontine neurons nucleus ≫ cytoplasmNot present
HD, n = 1StriatumCaudate neuronsNot present
DRPLA, n = 1CerebellumDentate and granule neurons nucleus ≫ cytoplasmNot present