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. 2019 Jan 4;23(3):1647–1656. doi: 10.1111/jcmm.13976

Table 1.

miRNAs modulating apoptosis and necroptosis pathways in ALS

miRNA Target molecule Regulatory effect Apoptosis/Necroptosis pathways Data in ALS
miR‐125b BCL2 Pro‐apoptotic35 Intrinsic apoptosis Up‐regulated in the CNS of SOD1G93A mice38, 39
7sl lncRNA Pro‐apoptotic37
Anti‐apoptotic36
p53 Extrinsic apoptosis
miR‐155 BCL2 Pro‐apoptotic35 Intrinsic apoptosis Up‐regulated in microglia of SOD1G93A mice39
Increased in the ALS spinal cord: fivefold in mice and twofold in humans40
Anti‐miR‐155 prolonged survival in SOD1 mice40, 41
Anti‐necroptotic77
RIP1 Necroptosis
miR‐365 and miR‐24 BCL2 Pro‐apoptotic43 Intrinsic aptoptosis Dysregulated in microglia ALS mice39
miR‐23a/b Apaf‐1 Anti‐apoptotic44 Intrinsic aptoptosis Increased in skeletal muscle of ALS patients; influence on mitochondrial function28
Pro‐apoptotic45
XIAP Intrinsic aptoptosis
miR‐27a/b Apaf‐1 Anti‐apoptotic44 Intrinsic apoptosis Reduced in serum of ALS patients46
Pro‐apoptotic68
FADD Extrinsic apoptosis
miR‐133a Caspase‐9 Anti‐apoptotic47 Intrinsic aptoptosis Reduced in in SOD1G93A mice muscle26
miR‐1 Unknown Anti‐apoptotic47 Intrinsic aptoptosis Reduced in in SOD1G93A mice muscle26
BCL2, HSP60, HSP70 Pro‐apoptotic48 Intrinsic aptoptosis
miR‐29a MCL‐1, P85a, CDC42 Pro‐apoptotic48, 50 Intrinsic aptoptosis Reduced in SOD1G93A mice muscle26
Increased in the lumbar spinal cord of ALS SOD1G3A mice49
miR‐375 cIAP?
cFLIP‐L?
p53, ELAVL4
Pro‐apoptotic69 Extrinsic apoptosis Reduced in ALS iPSCs‐derived MNs carrying FUS mutations71
Anti‐apoptotic70
miR‐34a BCL2 Pro‐apoptotic56, 57 Intrinsic apoptosis Decreased in SOD1G93A mice brainstem and spinal cord62
SIRT1
miR‐26 MTDH EZH2 Pro‐apoptotic59 Intrinsic and extrinsic apoptosis Decreased in SOD1G93A mice brainstem and spinal cord62
miR‐21 BCL2, TGFBR2, Pdcd4 Anti‐apoptotic65 Intrinsic apoptosis Increased in spinal cord and decreased in brainstem of SOD1G93A mice62
PPARα, FASL Extrinsic apoptosis
PTEN Intrinisc and extrinsic apoptosis

ALS: Amyotrophic lateral sclerosis; Apaf‐1: apoptotic protease activating factor‐1; CDC42: cell division control protein 42 homolog; cFLIP‐L: cellular FLICE (FADD‐like IL‐1β‐converting enzyme)‐inhibitory protein; cIAP: Cellular inhibitor of apoptosis 1; CNS: central nervous system; FADD: Fas‐associated protein with death domain; FASL: Fas Ligand; ELAVL4: ELAV‐like protein 4; EZH2: enhancer of zeste homolog 2; iPSCs: induced pluripotent stem cells; lncRNA: long noncoding RNA; miRNA: micro‐RNA; MCL‐1: induced myeloid leukaemia cell differentiation; MNs: motor neurons; MTDH: Metadherin; Pdcd4: programmed cell death 4; PPARα: peroxisome proliferator activated receptor alpha; PTEN: phosphatase and tensin homolog; RIP1: receptor‐interacting protein 1; SIRT1: silent information regulator 1; TGFBR2: transforming growth factor beta receptor II; XIAP: X‐linked inhibitor of apoptosis protein.