CHMP2B Recombinant Monoclonal Antibody

Code: CSB-RA131135A0HU

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Size:

50μl100μl

US$210

Quantity:

Species Reactivity: Human, Mouse

Application: ELISA, WB, IHC, FC

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Product Details
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Target Background

Product Details

Uniprot NO.

Q9UQN3

Target Names

CHMP2B

Alternative Names

ALS17 antibody; Charged multivesicular body protein 2b antibody; CHM2B_HUMAN antibody; CHMP family; member 2B antibody; CHMP2.5 antibody; CHMP2b antibody; Chromatin modifying protein 2b antibody; Chromatin-modifying protein 2b antibody; DMT1 antibody; hVps2-2 antibody; Vacuolar protein sorting 2; yeast; homolog of; B antibody; Vacuolar protein sorting 2-2 antibody; Vacuolar protein sorting-associated protein 2-2 antibody; VPS2 homolog B antibody; Vps2-2 antibody; VPS2B antibody

Species Reactivity

Human, Mouse

Immunogen

A synthesized peptide from human CHMP2B protein

Conjugate

Non-conjugated

Clonality

Monoclonal

Isotype

Rabbit IgG

Clone No.

5D4

Purification Method

Affinity-chromatography

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.

Form

Liquid
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.

Scope Application

ELISA Protocol Western Blotting(WB) Protocol Immunohistochemistry (IHC) Protocol Flow Cytometry (FC) Protocol

Recommended Dilution

Application	Recommended Dilution
WB	1:500-1:5000
IHC	1:50-1:200
FC	1:50-1:200

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.

Usage

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

Datasheet & COA

Antibody FAQs

Images

Western Blot
, Positive WB detected in:COLO205 whole cell lysate(30µg), A431 whole cell lysate(30µg), A549 whole cell lysate(30µg), NIH/3T3 whole cell lysate(30µg)
All lanes: CHMP2B antibody at 1:1000
Secondary
Goat polyclonal to rabbit IgG at 1/40000 dilution
Predicted band size: 24 kDa
Observed band size: 28 kDa
Exposure time：120s
IHC image of CSB-RA131135A0HU diluted at 1:100 and staining in paraffin-embedded human liver cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
Overlay Peak curve showing Hela cells stained with CSB-RA131135A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for 10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/1*10⁶cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 35min at 4℃.Control antibody (green line) was Rabbit IgG (1ug/1*10⁶cells) used under the same conditions. Acquisition of >10, 000 events was performed.

Description

CHMP2B plays a critical role in the endosomal sorting complexes required for transport (ESCRT) machinery, facilitating membrane remodeling during multivesicular body formation and autophagosome-lysosome fusion. Mutations in CHMP2B are directly linked to familial amyotrophic lateral sclerosis (ALS17) and frontotemporal dementia, making this protein a significant focus for researchers investigating neurodegenerative disease mechanisms and cellular quality control pathways.

This recombinant monoclonal antibody, generated from clone 5D4 in rabbit host, offers the reproducibility and sequence-defined consistency that demanding experimental workflows require. Unlike traditional hybridoma-derived antibodies, recombinant production ensures lot-to-lot uniformity, allowing you to maintain confidence in your results across extended studies and collaborative projects.

Validation across multiple platforms demonstrates this antibody's versatility in your research toolkit. Western blot analysis successfully detects CHMP2B in human cell lines including COLO205, A431, and A549, as well as mouse NIH/3T3 cells, confirming cross-species reactivity between human and mouse samples. The observed band size of 28 kDa runs slightly higher than the predicted 24 kDa molecular weight, a shift commonly attributed to post-translational modifications such as phosphorylation or the protein's inherent charge characteristics affecting gel migration. Immunohistochemistry validation in paraffin-embedded human liver cancer tissue demonstrates reliable performance in archival samples, while flow cytometry analysis in HeLa cells confirms utility for single-cell applications requiring intracellular staining.

Whether you are investigating ESCRT pathway dynamics, autophagy regulation, or the molecular basis of neurodegeneration, this antibody provides a dependable tool for signal transduction research across multiple experimental approaches.

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Target Background

Function(From Uniprot)

Probable core component of the endosomal sorting required for transport complex III (ESCRT-III) which is involved in multivesicular bodies (MVBs) formation and sorting of endosomal cargo proteins into MVBs. MVBs contain intraluminal vesicles (ILVs) that are generated by invagination and scission from the limiting membrane of the endosome and mostly are delivered to lysosomes enabling degradation of membrane proteins, such as stimulated growth factor receptors, lysosomal enzymes and lipids. The MVB pathway appears to require the sequential function of ESCRT-O, -I,-II and -III complexes. ESCRT-III proteins mostly dissociate from the invaginating membrane before the ILV is released. The ESCRT machinery also functions in topologically equivalent membrane fission events, such as the terminal stages of cytokinesis and the budding of enveloped viruses (HIV-1 and other lentiviruses). ESCRT-III proteins are believed to mediate the necessary vesicle extrusion and/or membrane fission activities, possibly in conjunction with the AAA ATPase VPS4.

Gene References into Functions

We directly link TDP-43 loss of function toxicity to two genes with rare amyotrophic lateral sclerosis and frontotemporal lobar degeneration-causing mutations, CHMP2B and ErbB4 PMID:27621269
these data indicate that the neuronal expression of human CHMP2B(intron5) in areas involved in motor and cognitive functions induces progressive motor alterations associated with dementia symptoms and with histopathological hallmarks reminiscent of both amyotrophic lateral sclerosis and frontotemporal dementia. PMID:27329763
Endogenous TMEM106B was partly sequestered in CHMP2B-positive structures. SNP T185 was more associated with CHMP2B than SNP S185, and it enhanced neurotoxicity caused by CHMP2B(Intron5) compared to S185-expressing cells. PMID:26651479
Study showed that mutant CHMP2B causes the pathological accumulation of endolysosomal components early in the frontotemporal dementia disease course PMID:26358247
Protein kinase CK2 alpha is involved in the phosphorylation of the ESCRT-III subunits CHMP3 and CHMP2B, as well as of VPS4B/SKD1, an ATPase that mediates ESCRT-III disassembly. PMID:24440309
Data indicate that knockdown of syntaxin 13 (syx13) further increased the cellular toxicity caused by muaant CHMP2B (CHMP2BIntron5) expression. PMID:24095276
CHMP2B immunoreactivity was increased in the dorsal motor nucleus of the vagus nerve in Parkinson's disease and incidental Lewy body disease brains PMID:22989140
These findings suggest that endosomal and autophagic pathway is associated with degradation or formation of alpha-synuclein aggregates in alpha-synucleinopathy. PMID:22947304
Subjects with CHMP2B mutation show cognitive changes dominated by executive dysfunctions, years before they fulfil diagnostic criteria of FTD. PMID:23142962
Direct link between disease-causing mutations and the cellular phenotype in cells originating from CHMP2B mutation patients with frontotemporal dementia. PMID:22786763
This study provided a better understanding of the cellular pathogenesis of neurodegenerative diseases associated with various missense mutations of CHMP2B as well as endocytic defects. PMID:22521643
CHMP2B polymerization scaffolds membranes in vivo represents a first step toward demonstrating its structural role during outward membrane deformation PMID:21926173
recent advances in our understanding of the molecular basis of CHMP2B mutations indicate that the mechanisms involved may be broadly relevant to neurodegenerative processes.[review] PMID:21222599
A novel heterozygous variant p.Ser194Leu (c.581C>T) is found in exon 6 of the CHMP2B gene in one male patient with pure frontotemporal lobar degeneration. PMID:20625756
The results of this study confirmed that mutations in CHMP2B are not a common cause of frontotemporal lobar degeneration. PMID:20412296
CHMP2B is required for spine growth. Taken together, these results demonstrate that a mutant ESCRT-III subunit linked to a human neurodegenerative disease can disrupt the normal pattern of spine development. PMID:20699355
analysis of CHMP2B mutations in lower motor neuron predominant amyotrophic lateral sclerosis PMID:20352044
The fusion of endosomes with lysosomes is required for neuronal function suggesting a pathogenic mechanism for frontotemporal dementia caused by CHMP2B mutations. PMID:20223751
CHMP2B can be used as a reliable marker for GVD in neurons of the AD hippocampus. PMID:20420883
there were no significant differences in the frequencies of the IVS4 + 44C/A, 1303C/A, 1254T/C and IVS15Ex16-16C/G polymorphism haplotypes in the patient cohorts (regardless of the degree of hepatic iron deposition) compared to the control cohort PMID:15223008
identify a mutation in CHMP2B, encoding a component of the endosomal ESCRTIII complex, and show that it results in aberrant mRNA splicing in tissue samples from affected members of family with autosomal dominant frontotermporal dementia PMID:16041373
Mutations in CHMP2B are a rare cause of familial FTLD and may be specific to the Danish pedigree. PMID:16431024
This studyidentified mutations (Q206H; I29V) in two patients with non-SOD1 ALS. PMID:16807408
CHMP2B mutations are not a cause of dementia in Dutch patients with familial and sporadic frontotemporal dementia. PMID:16941655
CHMP2B can be excluded as a susceptibility gene conferring risk to sporadic forms of frontotemporal dementia. PMID:16979267
These data suggest that C-truncating mutations in CHMP2B might underlie the pathogenic mechanism in frontotemporal lobar degeneration. PMID:17956895
data indicates that CHMP2B mutations are a rare cause of ALS, and no mutations were found in classic ALS phenotypes. PMID:18270236
These data suggest that SgIII, DMT-1 and HNP-1 are implicated in cell-mediated LDL oxidation. PMID:19150442
The specificity of DMT1 inhibition by 4 molecules in a cell line is reported. PMID:19179627
finding suggests that mutations in CHMP2B have widespread effects throughout the brain, leading to a neuro-anatomical signature distinct from other diseases in the frontotemporal lobar degeneration spectrum PMID:19202337
Divalent metal transporter 1 (DMT1) regulation by Ndfip1 prevents metal toxicity in human neurons. PMID:19706893

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Subcellular Location

Cytoplasm, cytosol. Late endosome membrane; Peripheral membrane protein.

Protein Families

SNF7 family

Tissue Specificity

Widely expressed. Expressed in brain, heart, skeletal muscle, spleen, kidney, liver, small intestine, pancreas, lung, placenta and leukocytes. In brain, it is expressed in cerebellum, cerebral cortex, medulla, spinal chord, occipital lobe, frontal lobe, t

Database Links

HGNC: 24537

UNIGENE: Hs.476930

KEGG: hsa:25978

STRING: 9606.ENSP00000263780

OMIM: 600795

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