Altered Gene Expression, Mitochondrial Damage and Oxidative Stress: Converging Routes in Motor Neuron Degeneration

Table 1

Genes involved in MNDs.

Gene	Protein	MND	Main known function
SOD1	Cu, Zn superoxide dismutase	ALS1	Antioxidant enzyme
ALS2	Alsin	ALS2	guanine nucleotide exchange factor for GTPases
SETX	Senataxin	ALS4	DNA/RNA metabolism and repair
SPG11	Spataxin	ALS5	Neuron differentiation and axonal transport
FUS/TLS	Fused in sarcoma	ALS6	RNA binding protein
VAPB	VAMP-associated protein B	ALS8	Trafficking between the endoplasmic reticulum and Golgi apparatus
TDP-43	TAR-DNA-binding protein-43	ALS9	DNA- and RNA-binding protein
ANG	Angiogenin	ALS10	Angiogenesis in response to hypoxia; possibly RNA metabolism
FIG4	PI(3,5)P(2)5-phosphatase	ALS11	Metabolism of phosphatidyl inositol bisphosphate and vesicle dynamic
OPTN	Optineurin	ALS12	Vesicular trafficking
nAChR	Neuronal nicotinic acetylcholine receptor	ALS	Glutamatergic pathway
CHMP2B	Charged multivesicular protein 2B	ALS	Chromatin-modifying protein/charged multivesicular body protein family
VCP	Valosin-containing protein	ALS	Membrane trafficking, organelle biogenesis, maturation of ubiquitin-containing autophagosomes
DAO	D-aminoacid oxidase	ALS	Oxidative deamination of D-aminoacid
UBQLN2	Ubiquilin2	ALS	Ubiquitin-proteasome response
Sig-1R	Sigma-1 receptor	ALS	ER chaperone, modulates calcium signaling through the IP3 receptor
C9ORF72	Unknown	ALS	Unknown
AR	Androgen receptor	SBMA	Androgen receptor
SMN	Survival Motor Neuron	SMA	RNA processing
GLE1	Nucleoporin GLE1	LCCS1	Export of mRNAs containing poly(A)