Catalog No.
RHN05103
Species reactivity
Human, Mouse, Rat, Monkey
Host species
Mouse
Isotype
IgG2b
Clonality
Monoclonal
Tested applications
ELISA: 1:10000, FCM: 1:200-1:400, IF: 1:200-1:1000, IHC: 1:200-1:1000, WB: 1:500-1:2000
Target
Guanine nucleotide exchange C9orf72, C9orf72
Concentration
1 mg/ml
Endotoxin level
Please contact with the lab for this information.
Purity
>95% as determined by SDS-PAGE.
Purification
Protein A/G purified from cell culture supernatant.
Accession
Q96LT7
Applications
ELISA, FCM, IF, IHC, WB
Form
Liquid
Storage buffer
0.01M PBS, pH 7.4, 0.05% Sodium Azide.
Stability and Storage
Use a manual defrost freezer and avoid repeated freeze-thaw cycles. Store at 4°C short term (1-2 weeks). Store at -20°C 12 months. Store at -80°C long term.
Clone ID
R3Z85
A patient-derived amyotrophic lateral sclerosis blood-brain barrier model for focused ultrasound-mediated anti-TDP-43 antibody delivery., PMID:39138578
Development and Preclinical Evaluation of a Copper-64-Labeled Antibody Targeting Glycine-Alanine Dipeptides for PET Imaging of C9orf72-Associated Amyotrophic Lateral Sclerosis/Frontotemporal Dementia., PMID:38751620
The MuSK agonist antibody protects the neuromuscular junction and extends the lifespan in C9orf72-ALS mice., PMID:38734896
MAPT H2 haplotype and risk of Pick's disease in the Pick's disease International Consortium: a genetic association study., PMID:38631765
Myeloid and lymphoid expression of C9orf72 regulates IL-17A signaling in mice., PMID:38295187
A fluid biomarker reveals loss of TDP-43 splicing repression in presymptomatic ALS-FTD., PMID:38278991
Validated assays for the quantification of C9orf72 human pathology., PMID:38191789
AAGGG repeat expansions trigger RFC1-independent synaptic dysregulation in human CANVAS Neurons., PMID:38168171
Altered plasma protein profiles in genetic FTD - a GENFI study., PMID:37968725
Poly-GA immunohistochemistry is a reliable tool for detecting C9orf72 hexanucleotide repeat expansions., PMID:37816685
A fluid biomarker reveals loss of TDP-43 splicing repression in pre-symptomatic ALS., PMID:36789434
Distribution of Lewy-related pathology in the brain, spinal cord, and periphery: the population-based Vantaa 85 + study., PMID:36510334
Therapeutic tools for familial ALS., PMID:36503675
Comprehensive evaluation of human-derived anti-poly-GA antibodies in cellular and animal models of C9orf72 disease., PMID:36454749
Gene Therapy in Amyotrophic Lateral Sclerosis., PMID:35805149
Identification of genetic risk loci and prioritization of genes and pathways for myasthenia gravis: a genome-wide association study., PMID:35074870
Antibody-Based Therapeutic Interventions for Amyotrophic Lateral Sclerosis: A Systematic Literature Review., PMID:34912191
A panel of CSF proteins separates genetic frontotemporal dementia from presymptomatic mutation carriers: a GENFI study., PMID:34838088
Passive Transfer of Blood Sera from ALS Patients with Identified Mutations Results in Elevated Motoneuronal Calcium Level and Loss of Motor Neurons in the Spinal Cord of Mice., PMID:34576165
NDST3 deacetylates α-tubulin and suppresses V-ATPase assembly and lysosomal acidification., PMID:34435379
Humoral response to neurofilaments and dipeptide repeats in ALS progression., PMID:34318620
Opinion: Independent third-party entities as a model for validation of commercial antibodies., PMID:34246180
C9orf72 deficiency promotes microglial-mediated synaptic loss in aging and amyloid accumulation., PMID:34133945
Gene therapy for ALS: A review., PMID:33839324
TFEB/Mitf links impaired nuclear import to autophagolysosomal dysfunction in C9-ALS., PMID:33300868
Epidemiological evidence for a hereditary contribution to myasthenia gravis: a retrospective cohort study of patients from North America., PMID:32948566
Antibody against TDP-43 phosphorylated at serine 375 suggests conformational differences of TDP-43 aggregates among FTLD-TDP subtypes., PMID:32778941
Development of disease-modifying drugs for frontotemporal dementia spectrum disorders., PMID:32203398
Cell-to-cell transmission of C9orf72 poly-(Gly-Ala) triggers key features of ALS/FTD., PMID:32175624
Generation and Characterization of Novel Monoclonal Antibodies Targeting p62/sequestosome-1 Across Human Neurodegenerative Diseases., PMID:32106300
Active poly-GA vaccination prevents microglia activation and motor deficits in a C9orf72 mouse model., PMID:31858749
Antibody Therapy Targeting RAN Proteins Rescues C9 ALS/FTD Phenotypes in C9orf72 Mouse Model., PMID:31831332
Synaptic localization of C9orf72 regulates post-synaptic glutamate receptor 1 levels., PMID:31651360
Implementation of an antibody characterization procedure and application to the major ALS/FTD disease gene C9ORF72., PMID:31612854
Lewy-related pathology exhibits two anatomically and genetically distinct progression patterns: a population-based study of Finns aged 85., PMID:31494694
C-terminal and full length TDP-43 specie differ according to FTLD-TDP lesion type but not genetic mutation., PMID:31266542
Increased prevalence of granulovacuolar degeneration in C9orf72 mutation., PMID:31144027
Complex phenotype in a C9ORF72-positive patient with high-titer anti-glutamic acid decarboxylase antibodies: neuroimmunology meets neurogenetics., PMID:31059159
C9orf72 and triplet repeat disorder RNAs: G-quadruplex formation, binding to PRC2 and implications for disease mechanisms., PMID:31048495
Concurrence of NMOSD and ALS in a patient with hexanucleotide repeat expansions of C9orf72., PMID:31007077
Misfolded SOD1 inclusions in patients with mutations in C9orf72 and other ALS/FTD-associated genes., PMID:30992335
Searching for Bacteria in Neural Tissue From Amyotrophic Lateral Sclerosis., PMID:30863279
The Association Between Frontotemporal Lobar Degeneration and Bullous Pemphigoid., PMID:30320585
New Antibody-Free Mass Spectrometry-Based Quantification Reveals That C9ORF72 Long Protein Isoform Is Reduced in the Frontal Cortex of Hexanucleotide-Repeat Expansion Carriers., PMID:30210275
Novel antibodies reveal presynaptic localization of C9orf72 protein and reduced protein levels in C9orf72 mutation carriers., PMID:30075745
Clinical and neuropathological features of ALS/FTD with TIA1 mutations., PMID:29216908
Neuron loss and degeneration in the progression of TDP-43 in frontotemporal lobar degeneration., PMID:28877758
Immunohistochemical detection of C9orf72 protein in frontotemporal lobar degeneration and motor neurone disease: patterns of immunostaining and an evaluation of commercial antibodies., PMID:28766957
Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degeneration., PMID:28420437
Poly-GP in cerebrospinal fluid links C9orf72-associated dipeptide repeat expression to the asymptomatic phase of ALS/FTD., PMID:28408402
For research use only. Not for human or drug use.
