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Fluorochrome-conjugated Monoclonal Antibodies for Alport's syndrome (Collagen IV coctail) Usage

Stain human cryostat sections by direct immunofluorescence

SGE-CFT45325 is prepared to stain human cryostat sections of renal and skin biopsy specimens by direct immunofluorescence.

How to stain human kidney cryostat sections
Just put 20-50 µl of the staining solution on cryostat sections, incubate them for 30 min at room temperature. After washing them with PBS, mount them and observe them with a fluorescence microscope. 

How to stain human skin cryostat sections
After acetone treatment of cryostat sections for 5 min, put 20-50 µl of the staining solution on them, and incubate them for 60 min in a moisture chamber at room temperature. After washing them with PBS, mount them and observe them with a fluorescence microscope. 


Cautions

  • Do not use stale cryostat sections.
  • Do not fix sections with any fixatives.
  • Do not dilute the staining solution.

Diagnosed as Alport's syndrome

In case that a section is not stained or stained in a mosaic pattern with FITC-anti-α5(IV) but the background staining of Texas Red-anti-α2(IV) is normally stained. The disease is diagnosed as Alport's syndrome.

Cautions

  • Human skin basement membrane is a thin membrane and sometimes staining of α5(IV) is very weak.
  • For accurate diagnosis, it is important to consider clinical symptoms. In case that the staining is not clear, it is necessary to make additional staining with other monoclonal antibodies against type IV collagen.

Product List

Product Name Cat# Quantity Price

Anti Collagen 4 cocktail for Alport's Syndrome

SGE-CFT-45325 1ML

¥ 55,000
$ 734
€ 550

Citation
  • Negative Staining for COL4A5 Correlates With Worse Prognosis and More Severe Ultrastructural Alterations in Males With Alport Syndrome
    Samar M. Said, Mary E.Fidler, Anthony M. Valeri, Brooke McCann, Wade Fiedler, Lynn D.Cornell, Mariam Priya Alexander, Ahmed M. Alkhunaizi, Anne Sullivan, Carl H. Cramer, Marie C. Hogan, Samih H. Nasr
    Kidney Int Rep. 2017 Jan;2(1):44-52. PMID:
  • 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.
    Malone AF, Funk SD, Alhamad T, Miner JH.
    Pediatr Nephrol. 2017 Jun;32(6):997-1003. PMID: 28013382
  • Alport syndrome and pregnancy: Good obstetric and nephrological outcomes in a pregnant woman with homozygous autosomal recessive Alport syndrome.
    Nishizawa Y, Takei T, Miyaoka T, Kamei D, Mochizuki T, Nitta K.
    J Obstet Gynaecol Res. 2016 Mar;42(3):331-5. PMID: 26628290
  • X-linked Alport syndrome caused by splicing mutations in COL4A5.
    Nozu K, Vorechovsky I, Kaito H, Fu XJ, Nakanishi K, Hashimura Y, Hashimoto F, Kamei K, Ito S, Kaku Y, Imasawa T, Ushijima K, Shimizu J, Makita Y, Konomoto T, Yoshikawa N, Iijima K.
    Clin J Am Soc Nephrol. 2014 Nov 7;9(11):1958-64. PMID: 25183659
  • 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.
    Matsubara S, Ueda Y, Takahashi H, Nagai T, Kuwata T, Muto S, Yamaguchi T, Takizawa T, Suzuki M.
    J Obstet Gynaecol Res. 2009 Dec;35(6):1109-14. PMID: 20144175
  • Collagen alpha5 and alpha2(IV) chain coexpression: analysis of skin biopsies of Alport patients.
    Patey-Mariaud de Serre N, Garfa M, Bessieres B, Noel LH, Knebelmann B.
    Kidney Int. 2007 Aug;72(4):512-6. PMID: 17554254
  • Autosomal dominant Alport's syndrome: study of a large Tunisian family.
    Kharrat M, Makni S, Makni K, Kammoun K, Charfeddine K, Azaeiz H, Jarraya F, Ben Hmida M, Gubler MC, Ayadi H, Hachicha J.
    Saudi J Kidney Dis Transpl. 2006 Sep;17(3):320-5. PMID: 16970251
  • In vivo expression of podocyte slit diaphragm-associated proteins in nephrotic patients with NPHS2 mutation.
    Zhang SY, Marlier A, Gribouval O, Gilbert T, Heidet L, Antignac C, Gubler MC.
    Kidney Int. 2004 Sep;66(3):945-54. PMID: 15327385
  • In vivo expression of putative LMX1B targets in nail-patella syndrome kidneys.
    Heidet L, Bongers EM, Sich M, Zhang SY, Loirat C, Meyrier A, Broyer M, Landthaler G, Faller B, Sado Y, Knoers NV, Gubler MC.
    Am J Pathol. 2003 Jul;163(1):145-55. PMID: 12819019

References
  • The NC1 domain of collagen IV encodes a novel network composed of the alpha 1, alpha 2, alpha 5, and alpha 6 chains in smooth muscle basement membranes.
    Borza DB, Bondar O, Ninomiya Y, Sado Y, Naito I, Todd P, Hudson BG.
    J Biol Chem. 2001 Jul 27;276(30):28532-40. PMID: 11375996
  • Epitope-defined monoclonal antibodies against type-IV collagen for diagnosis of Alport's syndrome.
    Kagawa M, Kishiro Y, Naito I, Nemoto T, Nakanishi H, Ninomiya Y, Sado Y.
    Nephrol Dial Transplant. 1997 Jun;12(6):1238-41. PMID: 9198058
  • Relationship between COL4A5 gene mutation and distribution of type IV collagen in male X-linked Alport syndrome. Japanese Alport Network.
    Naito I, Kawai S, Nomura S, Sado Y, Osawa G.
    Kidney Int. 1996 Jul;50(1):304-11. PMID: 8807602
  • Differential expression of two basement membrane collagen genes, COL4A6 and COL4A5, demonstrated by immunofluorescence staining using peptide-specific monoclonal antibodies.
    Ninomiya Y, Kagawa M, Iyama K, Naito I, Kishiro Y, Seyer JM, Sugimoto M, Oohashi T, Sado Y.
    J Cell Biol. 1995 Sep;130(5):1219-29. PMID: 7657706
  • Type IV collagen alpha 5 chain. Normal distribution and abnormalities in X-linked Alport syndrome revealed by monoclonal antibody.
    Yoshioka K, Hino S, Takemura T, Maki S, Wieslander J, Takekoshi Y, Makino H, Kagawa M, Sado Y, Kashtan CE.
    Am J Pathol. 1994 May;144(5):986-96. PMID: 8178947

To be used for research only. DO NOT use for human gene therapy or clinical diagnosis.