DES Recombinant Monoclonal Antibody

Code: CSB-RA571495A0HU

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Size:

50μl100μl

US$210

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Species Reactivity: Human, Mouse, Rat

Application: ELISA, WB, IHC, IF

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Product Details
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Target Background

Product Details

Uniprot NO.

P17661

Target Names

DES

Alternative Names

CMD1I antibody; CSM1 antibody; CSM2 antibody; DES antibody; DESM_HUMAN antibody; Desmin antibody; FLJ12025 antibody; FLJ39719 antibody; FLJ41013 antibody; FLJ41793 antibody; Intermediate filament protein antibody; OTTHUMP00000064865 antibody

Species Reactivity

Human, Mouse, Rat

Immunogen

A synthesized peptide from human DES protein

Conjugate

Non-conjugated

Clonality

Monoclonal

Isotype

Rabbit IgG

Clone No.

8E7

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 Immunofluorescence (IF) Protocol

Recommended Dilution

Application	Recommended Dilution
WB	1:500-1:5000
IHC	1:50-1:200
IF	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:Mouse Heart tissue lysate(30µg), Rat Heart lysate(30µg)
All lanes: Desmin antibody at 1:1000
Secondary
Goat polyclonal to rabbit IgG at 1/40000 dilution
Predicted band size: 54 kDa
Observed band size: 54 kDa
Exposure time：120s
IHC image of CSB-RA571495A0HU diluted at 1:100 and staining in paraffin-embedded human skeletal muscle tissue 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.
IHC image of CSB-RA571495A0HU diluted at 1:100 and staining in paraffin-embedded human prostate 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.
Immunofluorescence staining of HepG2 cell with CSB-RA571495A0HU at 1:50 , counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).

Description

Desmin serves as a critical intermediate filament protein that maintains the structural integrity of muscle cells, forming a network that connects myofibrils to each other and to the plasma membrane. As a key marker for muscle differentiation and a protein implicated in various myopathies and cardiac disorders, desmin detection is essential for researchers investigating muscle biology, cardiomyopathy mechanisms, and tissue development.

This recombinant monoclonal antibody, generated against a synthetic peptide derived from human desmin, offers the consistency and reproducibility that demanding research applications require. The recombinant format ensures sequence-defined specificity and eliminates the lot-to-lot variability often encountered with traditional hybridoma-derived antibodies, giving you confidence that your results will remain comparable across extended studies.

Validation across multiple experimental platforms demonstrates this antibody's versatility in your workflow. Western blot analysis of mouse heart tissue and rat heart lysate reveals clean detection of desmin at the expected 54 kDa molecular weight, confirming accurate target recognition. For tissue-based studies, immunohistochemistry has been validated in paraffin-embedded human skeletal muscle and prostate cancer tissue using citrate buffer antigen retrieval, producing reliable staining patterns. Immunofluorescence applications have been confirmed in HepG2 cells, enabling subcellular localization studies of desmin expression.

With demonstrated cross-species reactivity spanning human, mouse, and rat samples, this antibody supports comparative studies and translational research models. The broad application compatibility across ELISA, western blot, immunohistochemistry, and immunofluorescence makes it a practical choice for laboratories investigating signal transduction pathways, muscle pathologies, or tumor microenvironment characterization where desmin expression patterns provide meaningful biological insights.

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Target Background

Function(From Uniprot)

Muscle-specific type III intermediate filament essential for proper muscular structure and function. Plays a crucial role in maintaining the structure of sarcomeres, inter-connecting the Z-disks and forming the myofibrils, linking them not only to the sarcolemmal cytoskeleton, but also to the nucleus and mitochondria, thus providing strength for the muscle fiber during activity. In adult striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z-line structures. May act as a sarcomeric microtubule-anchoring protein: specifically associates with detyrosinated tubulin-alpha chains, leading to buckled microtubules and mechanical resistance to contraction. Contributes to the transcriptional regulation of the NKX2-5 gene in cardiac progenitor cells during a short period of cardiomyogenesis and in cardiac side population stem cells in the adult. Plays a role in maintaining an optimal conformation of nebulette (NEB) on heart muscle sarcomeres to bind and recruit cardiac alpha-actin.

Gene References into Functions

A novel mutation (c.679 C>T /p.R227C) in exon 3 of DES was identified and cosegregated with the affected members of a Chinese family with isolated Dilated cardiomyopathy (DCM) phenotypes (left ventricle and left atrial diameters). PMID:28171858
Desmin, Glial Fibrillary Acidic Protein, Vimentin, and Peripherin are type III intermediate filaments that have roles in health and disease [review] PMID:29196434
Phenotypic expression of a novel desmin gene mutation: hypertrophic cardiomyopathy followed by systemic myopathy. PMID:29167554
Targeted sequencing revealed trigenic mutations: c.700G>A/p.E234K in DES, c.2966G>A/p.R989H in MYPN, and c.5918G>C/p.R1973P in CACNA1C in a family of hypertrophic cardiomyopathy with early repolarization and short QT syndrome. PMID:28427417
demonstrate that the expression of mutant desmin causes disruption of the extrasarcomeric desmin cytoskeleton and extensive mitochondrial abnormalities regarding subcellular distribution, number and shape PMID:27393313
Mutation in the Core Structure of Desmin Intermediate Filaments Affects Myoblast Elasticity PMID:28793217
Data show that the filament elongation of both desmin and keratin K8/K18 proceeds very similar to that of vimentin. PMID:27304995
Cdk1-induced desmin phosphorylation is required for efficient separation of desmin-IFs and generally detected in muscular mitotic cells in vivo. PMID:27565725
Desmin, Lamin A/C, MMP9, and histone H4 were upregulated in the placental villi of women experiencing early pregnancy loss. PMID:26947931
Ile367Phe, Pro419Ser, and Arg415Glu mutations were associated with desminopathy causing cardiomyopathy in 4 families studied. PMID:26431784
Increasing desmin abnormalities were correlated with diastolic dysfunction progression. PMID:25732530
expression level of mutant versus wild-type desmin in mouse model as well as in skeletal muscle specimens derived from human R350P desminopathies; findings demonstrate missense-mutant desmin inflicts changes of the subcellular localization and turnover of desmin itself and of direct desmin-binding partners PMID:25394388
Results propose that the mutations affect desmin structure and cause its aberrant folding and subsequent aggregation, triggering disruption of myofibrils organization. PMID:25541946
identified disruption of the desmin system in gastrocnemius myofibers as an index of the myopathy and limitation of muscle function in patients with peripheral artery disease. PMID:25575565
The desmin intermediate filament network plays a major role in striated muscle development and maintenance by integrating and coordinating most cellular components necessary for proper mechanochemical signaling, organelle cross-talk, energy production and trafficking processes required for proper tissue homeostasis. [Review] PMID:25680090
Data suggest that loss of the desmin-p. A120D filament localization at the intercalated disk indicates its clinical arrhythmogenic potential. PMID:24200904
we describe a new mutation located in the coiled 1B domain of desmin and associated with a predominant cardiac involvement and a high degree of cardiac sudden death in a large Indian pedigree with 12 affected members PMID:24441330
Perfomed proteomic analysis on a transgenic mouse model of severe cardiac hypertrophy; compared data to dataset of heart failure found MYH7, IGFBP7, ANXA2, and DESM to be biomarker candidates for heart failure. PMID:23713052
autosomal recessive mutations in DES cause LGMD2 phenotype without features of myofibrillar myopathy. PMID:23687351
Data suggest that for some filament-forming desmin mutants, the molecular etiology of desminopathy results from subtle deficiencies in their association with nebulin, a major actin-binding filament protein of striated muscle. PMID:23615443
Sequencing of the desmin gene showed a splice-site mutation (IVS3+1G-->A), which was absent in 300 healthy control subjects. PMID:22484823
Phenotypic features in patients with desmin tail domain mutations are similar to those in patients with mutations localized in the 1B and 2B alpha-helical domains. PMID:23051780
The results of this study indicate that atrioventricular conduction block without cardiac structural abnormalities may be an intrinsic feature of disease associated with specific desmin mutation PMID:23036309
in the absence of skeletal muscle involvement suggestive of a desminopathy, the probability of DES mutations in ARVC is very low. These findings have important implications in the mutation screening strategy for patients with ARVC. PMID:23168288
The results of this study indicated that no cases showed missense mutations in the Desmin. PMID:22349865
Data show that calretinin and CK5/6 were positive in 100 and 64% of mesotheliomas, and 92 and 31% of reactive effusions, respectively, and desmin was negative in all malignant cases and positive in 85% of reactive effusions. PMID:23075894
A heterozygous C-to-T mutation in the desmin gene on chromosome 2q35 caused autosomal dominant myofibrillar myopathy with arrhythmogenic right ventricular cardiomyopathy 7 in a Swedish family. PMID:22395865
Frequent desmin (32%) and occasional CD34 (6%) expression are encountered in cellular fibrous histiocytoma. PMID:22775584
Data indicate that the interaction and co-localization of mutant and wild-type desmin proves the co-existence of heterogeneous filaments in living cells. PMID:22403400
analysis of CD105, CD31, alpha-SMA, vimentin and desmin expression on a series of normal human heart tissues varying between five and 33 weeks PMID:22395512
This study demonistrated that Patients carrying DES mutations presenting with myofibrillar myopathies or without muscle involvement, are at high risk of developing major cardiac complications. PMID:22153487
the expression of mutant desmin leads to increased mechanical stiffness, which results in excessive mechanical stress in response to strain and consecutively to increased mechanical vulnerability and damage of muscle cells. PMID:22386993
Ankrd1 and desmin may play important roles in airway smooth muscle cell homeostasis. PMID:22085644
49 mutations have been identified in the desmin gene, which alter the desmin filament assembly process through different molecular mechanisms and also its interaction with its protein partners. Review. PMID:21982405
these studies highlight the importance of desmin in maintaining cardiomyocyte structure and illustrate how disrupting this network can be deleterious to the heart--{REVIEW} PMID:21784990
study of patients with heart dilation of various origins; conclude A213V desmin substitution represents a rare polymorphism that plays role as predisposing factor resulting in maladaptive heart remodelling in presence of other pathological factors PMID:21842594
Novel mutations of desmin gene were linked with cardiomyopathy in patients from 5 Chinese families with desminopathy. PMID:20654101
Six novel mutations and one previously reported mutation in the desmin gene were identified in the patients PMID:20696008
the state of desmin-filament assembly is crucial for synemin anchorage and consequently might involve mechanical and functional stability of the cytoskeletal network PMID:21262226
study provides evidence on functional consequences of a novel mutation, N116S, identified in the desmin 1A segment of the rod domain for the development of arrhythmogenic right ventricular cardiomyopathy PMID:20829228
Case Report: present a rare case of desmin-related hypertrophic cardiomyopathy. Cardiac magnetic resonance imaging revealed fibrosis in the lateral wall of the left ventricle. PMID:21083940
mutations in MTM1 disrupted the MTM1-desmin complex, resulting in abnormal intermediate filament assembly and architecture in muscle cells PMID:21135508
Desmin mutations affects the localization of desmoplakin and plakophilin-2 at the intercalated disk suggesting a link between desmosomal cardiomyopathies (mainly affecting the right ventricle) and cardiomyopathies caused by desmin mutations. PMID:20423733
study of aggregation properties of desmin in vitro & aggregation state of desmin in homogenates of transfected cells; detected divergent assembly patterns for 3 different desmin missense mutations PMID:20448486
The Uruguayan family with severe cardiomyopathy carries an unusual deletion p.E114del within the 1A rod domain of desmin. PMID:20133133
the "tail" domain is responsible for attractive filament-filament interactions PMID:20171226
The "head" mutations in desmin proteins impacting on intermediate filament assembly properties and their competition for binding to cellular anchoring structures might explain part of the molecular mechanism that causes myhofibrillar myopathy disease. PMID:19763525
A localized effect of desmin on the structure of the cardiac intercalated disks might contribute to disease pathogenesis PMID:19879535
Data showed that the elevated expression of desmin was correlated with the severity and differentiation of CRC. PMID:19460759
structural and functional analysis of a new variant causing desmin-related myopathy PMID:11668632