Immunohistochemical staining of Desmin of human FFPE tissue followed by incubation with HRP labeled secondary and development with DAB substrate.

Desmin [D33] Antibody (cGMP).

Name: Desmin [D33] Antibody (cGMP).
Brand: enQuire Bio
Price: 135 USD
Availability: InStock

$ 135.00 – $ 455.00

Please Select Product Options Below To View The Catalog Number.

SKU: QIVD118
Species: Human
Tested Applications: IHC [IVD]
Available Conjugates:
Isotype: Mouse IgG1, kappa

Product Number	Description	Price
QIVD118-0.1ml	Size: 0.1 ml, Format: Concentrate	$ 135.00
QIVD118-0.5ml	Size: 0.5 ml, Format: Concentrate	$ 265.00
QIVD118-1ml	Size: 1 ml, Format: Concentrate	$ 455.00
QIVD118-6ml	Size: 6 ml, Format: Predilute	$ 215.00

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In Stock

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Datasheets and Documentation

Product Datasheet

Certificate of Analysis and Tags (Coming Soon)

SKU: QIVD118-0.1ml

Desmin General Information
Alternate Names

Molecular Weight
53.5 kDa
Chromosomal Location
q35 [chr: 2] [chr_start: 219418377] [chr_end: 219426739] [strand: 1]
Curated Database and Bioinformatic Data
Gene Symbol	DES
Entrez Gene ID	1674
RefSeq Protein Accession(s)	NP_001918
RefSeq mRNA Accession(s)	; NM_001927
RefSeq Genomic Accession(s)	NC_000002; NG_008043
UniProt ID(s)	P17661
PharmGKB ID(s)	PA27253
KEGG Gene ID(s)	hsa:1674
Associated Diseases (KEGG IDs)	Myopathy, myofibrillar, 1 (MFM1) [MIM:601419]: A form of myofibrillar myopathy, a group of chronic neuromuscular disorders characterized at ultrastructural level by disintegration of the sarcomeric Z disk and myofibrils, and replacement of the normal myofibrillar markings by small dense granules, or larger hyaline masses, or amorphous material. MFM1 is characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and accumulation of desmin-reactive deposits in cardiac and skeletal muscle cells. {ECO:0000269\|PubMed:10545598, ECO:0000269\|PubMed:10717012, ECO:0000269\|PubMed:10905661, ECO:0000269\|PubMed:11061256, ECO:0000269\|PubMed:11668632, ECO:0000269\|PubMed:12620971, ECO:0000269\|PubMed:12766977, ECO:0000269\|PubMed:14648196, ECO:0000269\|PubMed:14711882, ECO:0000269\|PubMed:14724127, ECO:0000269\|PubMed:15495235, ECO:0000269\|PubMed:15800015, ECO:0000269\|PubMed:16009553, ECO:0000269\|PubMed:16376610, ECO:0000269\|PubMed:16865695, ECO:0000269\|PubMed:17221859, ECO:0000269\|PubMed:18061454, ECO:0000269\|PubMed:19879535, ECO:0000269\|PubMed:20829228, ECO:0000269\|PubMed:22106715, ECO:0000269\|PubMed:22395865, ECO:0000269\|PubMed:23615443, ECO:0000269\|PubMed:23687351, ECO:0000269\|PubMed:25394388, ECO:0000269\|PubMed:27733623, ECO:0000269\|PubMed:28470624, ECO:0000269\|PubMed:9697706, ECO:0000269\|PubMed:9736733}. The disease is caused by mutations affecting the gene represented in this entry. Mutations in the DES gene are associated with a variable clinical phenotype which encompasses isolated myopathies, pure cardiac phenotypes (including dilated cardiomyopathy, restrictive cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy), cardiac conduction disease, and combinations of these disorders. If both cardiologic and neurologic features occur, they can manifest in any order, as cardiologic features can precede, occur simultaneously with, or follow manifestation of generalized neuromuscular disease (PubMed:19879535). {ECO:0000269\|PubMed:19879535}.; Cardiomyopathy, dilated 1I (CMD1I) [MIM:604765]: A disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. {ECO:0000269\|PubMed:10430757, ECO:0000269\|PubMed:24200904, ECO:0000269\|PubMed:26724190}. The disease is caused by mutations affecting the gene represented in this entry.; Neurogenic scapuloperoneal syndrome Kaeser type (Kaeser syndrome) [MIM:181400]: Autosomal dominant disorder with a peculiar scapuloperoneal distribution of weakness and atrophy. A large clinical variability is observed ranging from scapuloperoneal, limb grindle and distal phenotypes with variable cardiac or respiratory involvement. Facial weakness, dysphagia and gynaecomastia are frequent additional symptoms. Affected men seemingly bear a higher risk of sudden, cardiac death as compared to affected women. Histological and immunohistochemical examination of muscle biopsy specimens reveal a wide spectrum of findings ranging from near normal or unspecific pathology to typical, myofibrillar changes with accumulation of desmin. {ECO:0000269\|PubMed:17439987, ECO:0000269\|PubMed:25394388}. The disease is caused by mutations affecting the gene represented in this entry.
General Description of Desmin .
This antibody reacts with desmin protein of 53 kDa. Both striated as well as smooth muscle cells stain well with this antibody. The antibody shows a broad interspecific cross-reactivity, reacting with human.

Antibody (Suitable for clinical applications)

Sample Type: FFPE Patient Samples.

Tested Applications: IHC. Approved for In Vitro Diagnostic Procedures on FFPE tissues. For tissue collection recommendations, please see datasheet sent with product.

Application Notes

Specification	Recommendation
Recommended Dilution (Conc)	1:20-1:40
Pretreatment	Citrate Buffer pH 6.0
Incubation Parameters	30 min at Room Temperature

Prior to use, inspect vial for the presence of any precipitate or other unusual physical properties. These can indicate that the antibody has degraded and is no longer suitable for patient samples. Please run positive and negative controls simultaneously with all patient samples to account and control for errors in laboratory procedure. Use of methods or materials not recommended by enQuire Bio including change to dilution range and detection system should be routinely validated by the user.

Clonality: Monoclonal

Anti-Desmin Antibody Clone: D33

Host and Isotype: Mouse IgG1, kappa

Recommended Positive Control Sample: Leiomyoma

Cellular Localization of Antibody D33 Staining: Cytoplasmic

Buffer and Stabilizer: PBS with 1% BSA and 0.05% NaN₃

Antibody Concentration: Lot specific. Plese contact tech support for data.

Immunogen: BALB/C mice were immunized with purified desmin from human muscle.

Storage Conditions: This antibody should be stored refrigerated (2-8°C). This product should not be used past the expiration date printed on the vial.

Desmin Information for Pathologists

Summary:

53 kDa intermediate filament first described in 1976 (Proc Natl Acad Sci USA 1976;73:4344), present near the Z line in sarcomeres, present in cardiac, smooth and striated muscle (Wikipedia). Expressed before actin in myoblasts. Variable expression in myofibroblasts. Uses by pathologists Common stain used in panels for mesenchymal markers.

Common Uses By Pathologists:

Common stain used in panels for mesenchymal markers. Confirms myogenic origin of tissue / tumors. Helps differentiate smooth muscle tumors (desmin+) from GIST (c-kit+, almost always desmin-). In serous effusion, more common in reactive mesothelial cells (84%) than mesothelioma (8%) or carcinoma (2%, Am J Surg Pathol 2001;25:1405). Diagrams / tables

Limitations and Warranty

This antibody is manufactured in accordance with clinical good manufacturing practices in an ISO13485:2016 certified production facility. It is intended for multiple uses including in vitro diagnostic use and research use only applications. Please see vial label for expiration date. We strive to always deliver antibodies with a shelf life of at least two years.

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