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Juvenile Amyotrophic Lateral Sclerosis 2

Also Known As:

Amyotrophic Lateral Sclerosis 2, Juvenile; ALS, Juvenile; ALS2

Networked: 106 relevant articles (3 outcomes, 3 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)
2.	Motor Neuron Disease (Primary Lateral Sclerosis)
3.	Neurodegenerative Diseases (Neurodegenerative Disease)
4.	Muscle Spasticity (Spastic)
5.	Paralysis (Palsy)

Experts

1.	Hadano, Shinji: 15 articles (10/2021 - 07/2003)
2.	Otomo, Asako: 13 articles (10/2021 - 07/2003)
3.	Ikeda, Joh-E: 12 articles (03/2010 - 07/2003)
4.	Kunita, Ryota: 9 articles (03/2010 - 07/2003)
5.	Suzuki-Utsunomiya, Kyoko: 8 articles (11/2018 - 09/2004)
6.	Mizumura, Hikaru: 7 articles (05/2008 - 07/2003)
7.	Cai, Huaibin: 6 articles (07/2009 - 11/2006)
8.	Shim, Hoon: 5 articles (07/2009 - 11/2006)
9.	Mitsui, Shun: 4 articles (10/2021 - 01/2016)
10.	Ono, Suzuka: 4 articles (10/2021 - 01/2016)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Juvenile Amyotrophic Lateral Sclerosis 2:

1.	Proteins (Proteins, Gene)FDA Link 10/19/2022 - "It is transmitted with a recessive pattern and originates from mutations of the ALS2 gene, encoding for the protein alsin and involved in differentiation and maintenance of the upper motoneuron. " 01/01/2022 - "ALS2 pathogenic variants are distributed widely across the entire coding sequence and mostly result in a loss of protein function. " 01/01/2018 - "This boy carries a heterozygous missense variant in KIF5A p.(Glu755Lys), inherited from the father, and a homozygous missense variant in the alsin protein encoded by the ALS2 gene p.(Pro192Leu). " 01/01/2016 - "Since SQSTM1 loss is more detrimental to SOD1H46R mice than lack of ALS2, the selective accumulation of such aggregates in neurons might be more insulting than the biochemically-detectable insoluble proteins. " 01/01/2016 - "Notably, absence of SQSTM1 rather suppressed the mutant SOD1-dependent accumulation of insoluble polyubiquitinated proteins, while ALS2 loss enhanced it. "
2.	Peptide Hydrolases (Proteases)FDA Link 04/01/2019 - "albicans displayed genes related to adhesion and proteinases (ALS2, ALS3, SAP1, and SAP3). " 01/01/2011 - "By digesting isolated cell walls with Sap9 or Sap10, we identified the covalently linked cell wall proteins (CWPs) Cht2, Ywp1, Als2, Rhd3, Rbt5, Ecm33, and Pga4 as in vitro protease substrates. " 03/29/2023 - "Using the improved protocol, we screened and compared the antibacterial activity of chimeric lysin candidates and verified the relatively strong activity associated with the CHAP (cysteine, histidine-dependent amidohydrolase/peptidase) domain of secretory antigen SsaA-like protein (ALS2). "
3.	HerbicidesIBA 12/01/2014 - "Although the Als1 and Als2 genes are unlinked, the combination of these two mutations is synergistic for improved tolerance of soybeans to ALS-inhibiting herbicides." 09/01/2013 - "Thus, the accession having an Asp376Glu mutation in ALS2 was highly resistant to the three SU herbicides and moderately resistant to BPS, but was not substantially resistant to IMQ. " 12/01/2014 - "Molecular and phenotypic characterization of Als1 and Als2 mutations conferring tolerance to acetolactate synthase herbicides in soybean." 09/01/2013 - "Schoenoplectus juncoides, a noxious weed for paddy rice, is known to become resistant to sulfonylurea (SU) herbicides by a target-site mutation in either of the two acetolactate synthase (ALS) genes (ALS1 and ALS2). " 02/01/2021 - "The subsequent herbicide sensitivity assay showed that the SD-06 population (with ALS1 P197T and ALS2 W574L mutations) exhibited cross-resistance to all ALS inhibitors tested, but was sensitive to MCPA-Na, fluroxypyr, carfentrazone-ethyl and bipyrazone. "
4.	Glutamate Receptors (Glutamate Receptor)IBA 07/24/2009 - "Consequently, a significant increase in endosome/lysosome-dependent degradation of internalized glutamate receptors was observed in ALS2(-/-) neurons. " 11/08/2006 - "In support of the physiological interaction between alsin and GRIP1, the subcellular distribution of GRIP1 was altered in ALS2(-/-) spinal motor neurons, which correlates with a significant reduction of AMPA-type glutamate receptor subunit 2 (GluR2) at the synaptic/cell surface of ALS2(-/-) neurons. " 11/08/2006 - "The decrease of calcium-impermeable GluR2-containing AMPA receptors at the cell/synaptic surface rendered ALS2(-/-) neurons more susceptible to glutamate receptor-mediated neurotoxicity. "
5.	Cysteine (L-Cysteine)FDA Link 03/29/2023 - "Using the improved protocol, we screened and compared the antibacterial activity of chimeric lysin candidates and verified the relatively strong activity associated with the CHAP (cysteine, histidine-dependent amidohydrolase/peptidase) domain of secretory antigen SsaA-like protein (ALS2). " 06/01/2006 - "This homozygous G669A mutation in exon 4 is predicted to result in a tyrosine substitution at cysteine 156 of the RCC1 (regulator of chromatin condensation)-like domain, encoding a putative guanine exchange factor for Ran guanosine triphosphatase, leading to a loss of ALS2 function due to instability of mutant protein. "
6.	AntigensIBA 10/05/2004 - "We characterized an antibody raised against a peptide derived from the ALS2 protein, the product of a gene linked to recessive juvenile forms of amyotrophic lateral sclerosis, defining the ALS2 RLD-derived antigen (ARDA). " 03/29/2023 - "Using the improved protocol, we screened and compared the antibacterial activity of chimeric lysin candidates and verified the relatively strong activity associated with the CHAP (cysteine, histidine-dependent amidohydrolase/peptidase) domain of secretory antigen SsaA-like protein (ALS2). "
7.	alpha- Amino- 3- hydroxy- 5- methyl- 4- isoxazolepropionic Acid (AMPA)IBA 11/08/2006 - "In support of the physiological interaction between alsin and GRIP1, the subcellular distribution of GRIP1 was altered in ALS2(-/-) spinal motor neurons, which correlates with a significant reduction of AMPA-type glutamate receptor subunit 2 (GluR2) at the synaptic/cell surface of ALS2(-/-) neurons. "
8.	Histidine (L-Histidine)FDA Link 03/29/2023 - "Using the improved protocol, we screened and compared the antibacterial activity of chimeric lysin candidates and verified the relatively strong activity associated with the CHAP (cysteine, histidine-dependent amidohydrolase/peptidase) domain of secretory antigen SsaA-like protein (ALS2). "
9.	AmidohydrolasesIBA 03/29/2023 - "Using the improved protocol, we screened and compared the antibacterial activity of chimeric lysin candidates and verified the relatively strong activity associated with the CHAP (cysteine, histidine-dependent amidohydrolase/peptidase) domain of secretory antigen SsaA-like protein (ALS2). "
10.	Superoxide Dismutase-1IBA 10/01/2007 - "Previous in vitro studies suggest that over-expression of ALS2 protects cells from mutant Cu/Zn superoxide dismutase (SOD1)-induced cytotoxicity. " 03/22/2010 - "Recently, it has been shown that ALS2 plays a role in neuroprotection against MND-associated pathological insults, such as toxicity induced by mutant Cu/Zn superoxide dismutase (SOD1). " 01/01/2008 - "ALS animal models in this review include strains of rodents that are transgenic for superoxide dismutase 1 (SOD1), ALS2 knockout mice, and mice that are transgenic for cytoskeletal abnormalities. " 07/01/2007 - "Further, a neuroprotective role for ALS2 against cytotoxicity; i.e., the mutant Cu/Zn-superoxide dismutase 1 (SOD1)-mediated toxicity, oxidative stress, and excitotoxicity, has recently been implied. " 03/01/2008 - "We recently demonstrated that activation of Akt3 by the autosomal-recessive familial amyotrophic lateral sclerosis (ALS)-linked gene 2 (ALS2) product, alsinLF, led to the suppression of motoneuronal death induced by familial ALS-related mutant superoxide dismutase-1 (SOD1). "