SKU: WP4131 Categories: ,

CI072 rabbit pAb

Price range: $268.00 through $328.00

SKU: WP4131-100
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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

Size50μL/100μL
TargetC9orf72
ReactivityHuman, Mouse, Rat
ApplicationsWB
MW(Calculated)53kD
MW(Observed)
Host SpeciesRabbit
IsotypeIgG
Conjugate/ModificationUnmodified
Recommended Dilution RatioWB 1:500-2000
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
PurificationThe antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Storage-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration1 mg/ml
ClonalityPolyclonal
Clone Number
ImmunogenSynthesized peptide derived from human CI072 AA range: 188-238
SpecificityThis antibody detects endogenous levels of CI072 at Human/Mouse/Rat
Gene NameC9orf72
Protein NameCI072
Other Name
SpeciesHuman
Gene ID203228
UniprotQ96LT7,
Species.1Mouse
Gene ID.1
Uniprot.1Q6DFW0,
Species.2Rat
Gene ID.2313155
Uniprot.2Q66HC3
Cellular LocalizationNucleus . 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 ExpressionBoth 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_pathwayCellular 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
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