Carbonic Anhydrase I modifies SOD1-induced motor neuron toxicity in Drosophila via ER stress pathway

Abstract

AAZ: Acetazolamide; ALS: Amyotrophic Lateral Sclerosis; FALS: Familial Amyotrophic Lateral Sclerosis; SALS: Sporadic Amyotrophic Lateral Sclerosis; ASK1: Apoptosis Signal Regulating Kinase 1; ATF4: Activating Transcription Factor 4; BSA: Bovine Serum Albumin; CA: Carbonic Anhydrase; CA1: Carbonic Anhydrase I; CAH1: Drosophila Carbonic Anhydrase form 1; CAH2: Drosophila Carbonic Anhydrase form 2; hCA1: Human Carbonic Anhydrase 1; CMT1A: Charcot-Marie-Tooth type 1A; DDS: Direct Digital Synthesis; Derlin-1: Degradation in Endoplasmic Reticulum Protein 1; dDerlin-1: Drosophila degradation in Endoplasmic Reticulum Protein 1; hDerlin-1: human Degradation in Endoplasmic Reticulum Protein 1; DMSO: Dimethyl Sulfoxide; dsod: Drosophila copper/zinc superoxide dismutase gene; E106I: Human CA1 mutant with a mutation in the amino acid at position 106 from glutamic acid (E) to isoleucine (I); eIF2\uf061: eukaryotic translation Initiation Factor 2 subunit alpha; p-eIF2\uf061: phosphorylated eukaryotic translation Initiation Factor 2 subunit alpha; ER: Endoplasmic Reticulum; FUS: Fused in Sarcoma, an RNA-binding protein; G93A: Human copper/zinc superoxide dismutase mutant with a mutation in the amino acid at the position 93 from glycine (G) to alanine (A); GFP: Green Fluorescent Protein; HNPP: Hereditary Neuropathy with liability to Pressure Palsies; Hsp70: The 70 kDa heat shock protein; JNK: c-Jun N-terminal Kinases; p-JNK: phosphorylated c-Jun N-terminal Kinases; PFN1: Pro ilin 1; PMP22: Peripheral Myelin Protein-22; SOD1: Copper/Zinc superoxide Dismutase; SOD1G85R: Human copper/zinc superoxide dismutase mutant with a mutation in the amino acid at the position 85 from glycine (G) to arginine (R); dSOD1: Drosophila Copper/Zinc Superoxide Dismutase; hSOD1: human Copper/Zinc Superoxide Dismutase; TBST: Tris-Buffered Saline, pH 7.5, 0.05% Tween 20; TDP-43: TAR DNA-Binding Protein; TRAF2: TNF Receptor-Associated Factor 2; XBP1: X-box Binding Protein 1; CA1 lies: Transgenic lies expressing human CA1 in motor neurons; E106I lies: Transgenic lies expressing human CA1 mutant E106I in motor Abstract