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. Author manuscript; available in PMC: 2015 Aug 21.
Published in final edited form as: Nat Neurosci. 2013 Dec 26;17(1):17–23. doi: 10.1038/nn.3584

Table 1. Genes causing ALS.

Values represent the percentage of ALS explained by each gene in Caucasian populations. Relevant references are provided in the main text.

Gene Location Inheritance Percentage explained Putative protein function

Familial ALS Sporadic ALS
TARDBP 1p36 AD 4 1 RNA metabolism
SQSTM1 5q35 AD 1 <1 Ubiquitination; autophagy
C9ORF72 9p21 AD 40 7 DENN protein
VCP 9p13 AD 1 1 Proteasome; vesicle trafficking
OPTN 10p13 AR & AD <1 <1 Vesicle trafficking
FUS 16p11 AD & AR 4 1 RNA metabolism
PFN1 17p13 AD <1 <1 Cytoskeletal dynamics
SOD1 21q22 AD & AR 12 1–2 Superoxide metabolism
UBQLN2 Xp11 XD <1 <1 Proteasome

AD = autosomal dominant; AR = autosomal recessive; XD = X-linked dominant; ALS = amyotrophic lateral sclerosis, and DENN = Differentially Expressed in Normal and Neoplasia.