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Alport syndrome is an inherited disease characterized by the pathological absence or reduction of the collagen α5(IV) chain in glomerular basement membrane (GBM), tubular basement membrane (TBM) and Bowman's capsular basement membrane. Anti-Collagen 4 Cocktail mAb (cat no. SGE-CFT45325) is a cocktail of three different rat mAbs for easy and rapid staining of human renal and skin biopsy sections to distinguish Alport syndrome from normal tissue. It comprises two different FITC-conjugated mAbs (clones H53 and B51) that reveal the Alport-affected α5(IV) chain in glomerular, tubular and Bowman's capsular BMs and an internal positive control Texas Red-conjugated mAb (clone H25) that targets α2(IV) to reveal endothelial basement membrane structure
Product specifications
| Specification |
Detail |
| Anti-Collagen 4 Cocktail mAb (Clones H53, B51, H25) |
| Cat No |
SGE-CFT45325 |
| Application |
Staining of human cryostat sections by direct immunofluorescence (acid-urea treatment not required) |
| Quantity |
1.0 ml |
| Appearance |
Solution, 0.1% NaN3 as preservative |
| Purification |
Affinity chromatography |
| Fluorescent labeling |
H53 and B51, FITC; H25, Texas Red |
| Clone names |
H53 (rat IgG2a/kappa), B51 (rat IgG2a) and H25 (rat IgG1/kappa) |
| Specificity |
H53 is specific to imperfection III of α5(IV) (Reference 1); B51 is specific to NC1 domain of α5(IV) (Reference 2); H25 is specific to imperfection XIII of α2(IV) (Reference 1). |
| Antibodies preparation |
Monoclonal antibodies were raised in rats against synthetic peptides and the native NC1 domain of type IV collagen. |
| Storage |
In the dark at 2-4°C, or below -30°C. Stable at these conditions for several years |
| Source |
Shigei Medical Research Institute, 2117 Yamada, Okayama 701-0202, Japan; TEL: +81-86-282-3113; FAX: +81-86-282-3115; E-mail: inst@shigei.or.jp |
References
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Kagawa M et al.
Epitope-defined monoclonal antibodies against type-IV collagen for diagnosis of Alport syndrome.
Nephrol. Dial. Transplant. 12: 1238-1241 (1997) (PMID: 9198058)
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Borza DB et al.
The NCI domain of collagen IV encodes a novel network composed of the α1, α2, α5, and α6 chains in smooth muscle basement membranes.
J. Biol. Chem. 276: 28532-28540 (2001) (PMID: 11375996)
-
Sado et al.
Establishment by the rat lymph node method of epitope-defined monoclonal antibodies recognizing the six different α chains of human type IV collagen.
Histochem. Cell Biol. 104: 267-275 (1995) (PMID: 8548560)
-
Yoshioka K et al.
Type IV collagen α5 chain: Normal distribution and abnormalities in X-linked Alport syndrome revealed by monoclonal antibody.
Am. J. Pathol. 144: 986-996 (1994) (PMID: 8178947)
-
Ninomiya Y et al.
Differential expression of two basement membrane collagen genes, COL4A6 and COL4A5, demonstrated by immunofluorescence staining using peptide-specific monoclonal antibodies.
J. Cell Biol. 130: 1219-1229 (1995) (PMID: 7657706)
-
Naito I et al.
Relationship between COL4A5 gene mutation and distribution of type IV collagen in male X-linked Alport syndrome.
Kidney Int. 50: 304-311 (1996) (PMID: 8807602)
Citations
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Bu L et al.
Somatic Mosaicism in a Male Patient With X-linked Alport Syndrome.
Kidney Int Rep. 14(4): 1031-1035 (2019) (PMID: 31312776)
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Samar M et al.
Negative Staining for COL4A5 Correlates With Worse Prognosis and More Severe Ultrastructural Alterations in Males With Alport Syndrome.
Kidney Int Rep. 2(1): 44-52 (2017) (PMID: 29142939)
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Malone AF et al.
Functional assessment of a novel COL4A5 splice region variant and immunostaining of plucked hair follicles as an alternative method of diagnosis in X-linked Alport syndrome.
Pediatr Nephrol. 32(6): 997-1003 (2017) (PMID: 28013382)
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Nozu K et al.
X-linked Alport syndrome caused by splicing mutations in COL4A5.
Am Soc Nephrol. 9(11): 1958-64 (2014) (PMID: 25183659)
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Matsubara S et al.
Pregnancy complicated with Alport syndrome: a good obstetric outcome and failure to diagnose an infant born to a mother with Alport syndrome by umbilical cord immunofluorescence staining.
Obstet Gynaecol Res. 35(6): 1109-14 (2009) (PMID: 20144175)
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Patey-Mariaud de Serre N et al.
Collagen alpha5 and alpha2(IV) chain coexpression: analysis of skin biopsies of Alport patients.
Kidney Int. 72(4): 512-6 (2007) (PMID: 17554254)
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Kharrat M et al.
Autosomal dominant Alport's syndrome: study of a large Tunisian family.
Kidney Dis Transpl. 17(3): 320-5 (2006) (PMID: 16970251)
