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Table 1 PolyQ-expansion diseases [1],[2],[17]

From: Pathogenic polyglutamine expansion length correlates with polarity of the flanking sequences

Disease

Protein

Localization

Qth

NPI

CPI

EPI

Neuropathology

HD

Htt

Cytoplasm

35

7.925

-2.047

5.878

Striatum, Cortex

SBMA

AR

Nucleus, Cytoplasm

38

-3.658

19.475

15.817

Spinal cord, Brain stem

DRPLA

ATN1

Nucleus, Cytoplasm

49

53.530

36.706

90.236

Cerebellum, Basal ganglia

SCA1

Atxn1

Nucleus

39

35.109

25.323

60.432

Cerebellum, Brain stem

SCA2

Atxn2

Cytoplasm

32

13.614

-2.222

11.392

Cerebellum, Brain stem

SCA3

Atxn3

Nucleus, Cytoplasm

54

20.552

12.979

33.531

Ventral pons, Substantia nigra

SCA6

CACN1A

Plasma Membrane

20

-

-

-

Cerebellum

SCA7

Atxn7

Nucleus, Cytoplasm

37

19.309

-2.047

17.262

Cerebellum, Brain stem, Spinal cord

SCA17

TBP

Nucleus

47

26.816

-3.758

23.058

Cerebellum, Striatum

  1. Footnote: Qth - pathogenic polyQ-expansion threshold; NPI - amino terminal polarity index; CPI - carboxy-terminal polarity index; EPI - total edge polarity index.