Name: U-Blot® C9orf72 Rabbit mAb
SKU: WR5276
Price: 268 USD
Availability: InStock

U-Blot® C9orf72 Rabbit mAb

Ucallm_WR5276-50

Price range: $268.00 through $328.00

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100μl

50μl

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SKU: WR5276-100

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Product details

Background:The protein encoded by this gene plays an important role in the regulation of endosomal trafficking, and has been shown to interact with Rab proteins that are involved in autophagy and endocytic transport. Expansion of a GGGGCC repeat from 2-22 copies to 700-1600 copies in the intronic sequence between alternate 5' exons in transcripts from this gene is associated with 9p-linked ALS (amyotrophic lateral sclerosis) and FTD (frontotemporal dementia) (PMID: 21944778, 21944779). Studies suggest that hexanucleotide expansions could result in the selective stabilization of repeat-containing pre-mRNA, and the accumulation of insoluble dipeptide repeat protein aggregates that could be pathogenic in FTD-ALS patients (PMID: 23393093). Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2016],

Specifications

Size	50μL/100μL
Target	C9orf72
Reactivity	Human, Mouse, Rat
Applications	WB, IHC, IF, ELISA
MW(Calculated)	54kD
MW(Observed)	55kD
Host Species	Rabbit
Isotype	IgG,Kappa
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	IHC 1:500-1:2000; WB 1:1000-1:20000; IF 1:200-1:1000; ELISA 1:5000-1:20000; / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004)
Formulation	PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
Purification	Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography.
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
Clonality	Monoclonal
Clone Number	RM1521R
Immunogen	The specific immunogen used to produce this antibody is proprietary information.
Specificity	Endogenous
Gene Name	C9orf72
Protein Name	CI072
Other Name
Species	Human
Gene ID	203228
Uniprot	Q96LT7,
Species.1	Mouse
Gene ID.1
Uniprot.1	Q6DFW0,
Species.2	Rat
Gene ID.2	313155
Uniprot.2	Q66HC3
Cellular Localization	Nucleus . Cytoplasm . Cytoplasm, P-body . Cytoplasm, Stress granule . Endosome . Lysosome . Cytoplasmic vesicle, autophagosome . Secreted . Cell projection, axon . Cell projection, growth cone . Perikaryon . Detected in the cytoplasm of neurons from brain tissue (PubMed:21944778). Detected in the nucleus in fibroblasts (PubMed:21944779). During corticogenesis, transitions from being predominantly cytoplasmic to a more even nucleocytoplasmic distribution (By similarity). .; [Isoform 1]: Perikaryon . Cell projection, dendrite . Cell junction, synapse, presynapse . Cell junction, synapse, postsynapse . Expressed diffusely throughout the cytoplasm and dendritic processes of cerebellar Purkinje cells. Also expressed diffusely throughout the cytoplasm of spinal motor neurons. .; [Isoform 2]: Nucleus membrane; Peripheral membrane protein . Nucleus . Detected at the nuclear membrane of cerebellar Purkinje cells and spinal motor neurons. Also shows diffuse nuclear expression in spinal motor neurons. .
Tissue Expression	Both isoforms are widely expressed, including kidney, lung, liver, heart, testis and several brain regions, such as cerebellum. Also expressed in the frontal cortex and in lymphoblasts (at protein level).
Signaling_pathway	Cellular Processes >> Transport and catabolism >> Autophagy - animal;Human Diseases >> Neurodegenerative disease >> Amyotrophic lateral sclerosis;Human Diseases >> Neurodegenerative disease >> Pathways of neurodegeneration - multiple diseases
Research Areas	>>Autophagy - animal; / >>Amyotrophic lateral sclerosis; / >>Pathways of neurodegeneration - multiple diseases
Function