货号：AB733

品牌：Merck Millipore

规格：1mL

目录价：￥4315.00

市场价格：￥3667.75

会员价格：￥3452.00

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Tamm-Horsfall Glycoprotein Antibody | AB733 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: One arc by 2D-IEP against Tamm-Horsfall glycoprotein (1 mg/mL). No precipitin line by IEP against normal human plasma. Identity verified by double immunodiffusion. View All» Immunogen: Highly purified human Tamm-Horsfall glycoprotein (uromucoid) from urine; >95% pure by SDS-PAGE. View All» Species Reactivity: Human View All» Application Notes: IEP: 100 μL antiserum vs. 5 μL uromucoid (50 mg/L) RID and Rocket RID: 10 μL antiserum/cm2 in gel vs. 5 μL uromucoid (50 mg/L), NT - 1/5 Double diffusion: 10 μL antiserum vs. 3 μL uromucoid (50 mg/L) *Note: The use of 3% PEG 6000 with 1.2% agarose in a suitable buffer (such as TBE or Tris-barbital pH >8.2) is recommended for the above applications. Immunohistochemistry Optimal working dilution must be determined by the end user. View All» Presentation: In glycine buffered saline, pH 7.4 containing 0.1% sodium azide, 0.1% E-Amino-n-Caproic Acid, 0.01% Benzamidine and 1 mM Ethylenediaminetetraacetic acid as preservatives. View All» Storage Conditions: Maintain at 2-8°C in undiluted aliquots for up to 12 months. View All» UniProt Number: P07911 View All» Entrez Gene Number:

• NM_001008389.1

• NM_003361.2
  View All» Gene Symbol:
  • UMOD

  • THP

  • THGP

  • HNFJ

  • ADMCKD2

  • uromucoid

  • uromodulin

  • MCKD2

  • FJHN
    View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications:
    • Immunodiffusion

    • Western Blotting

    • Immunohistochemistry
      View All» Entrez Gene Summary: This gene encodes uromodulin, the most abundant protein in normal urine. Its excretion in urine follows proteolytic cleavage of the ectodomain of its glycosyl phosphatidylinosital-anchored counterpart that is situated on the luminal cell surface of the loop of Henle. Uromodulin may act as a constitutive inhibitor of calcium crystallization in renal fluids. Excretion of uromodulin in urine may provide defense against urinary tract infections caused by uropathogenic bacteria. Defects in this gene are associated with the autosomal dominant renal disorders medullary cystic kidney disease-2 (MCKD2) and familial juvenile hyperuricemic nephropathy (FJHN). These disorders are characterized by juvenile onset of hyperuricemia, gout, and progressive renal failure. While several transcript variants may exist for this gene, the full-length natures of only two have been described to date. These two represent the major variants of this gene and encode the same isoform. View All» UniProt Summary: FUNCTION: SwissProt: P07911 # Not known. May play a role in regulating the circulating activity of cytokines as it binds to IL-1, IL-2 and TNF with high affinity.SIZE: 640 amino acids; 69761 Da SUBCELLULAR LOCATION: Cell membrane; Lipid-anchor, GPI-anchor. Secreted. Note=Secreted after cleavage in the urine.TISSUE SPECIFICITY: Synthesized by the kidneys and is the most abundant protein in normal human urine.DISEASE: SwissProt: P07911 # Defects in UMOD are the cause of familial juvenile hyperuricemic nephropathy (HNFJ) [MIM:162000]. HNFJ is a heritable autosomal dominant renal disease characterized by juvenil onset of hyperuricaemia, polyuria, progressive renal failure, and gout. The disease is associated with interstitial pathological changes resulting in fibrosis. & Defects in UMOD are the cause of medullary cystic kidney disease 2 (MCKD2) [MIM:603860]. MCKD2 and HNFJ constitute a group of heritable renal diseases with a common mode of transmission (autosomal dominant) and shared features including polyuria, hyperuricaemia, progressive renal failure, and gout. Both diseases are associated with interstitial pathological changes resulting in fibrosis. While corticomedullary cysts are well documented in MCKD2, their presence in HNFJ is not well documented. The primary clinical features of MCKD2 and HNFJ vary in presence and severity, complicating the diagnosis of these conditions, particularly in milder cases. Both diseases are considered to be allelic diseases. & Defects in UMOD are a cause of glomerulocystic kidney disease with hyperuricemia and isosthenuria [MIM:609886]. Glomerulocystic kidney disease (GCKD) and medullary cystic disease/familial juvenile hyperuricemic nephropathy (MCKD/HNFJ) are two distinct renal disorders that share some common clinical features. The former is characterized by a cystic dilatation of Bowman's space and a collapse of glomerular tuft. Familial GCKD can be associated with either hypoplastic or normal sized kidneys. A GCKD clinical variant presents the association with hyperuricemia due to low fractional excretion of uric acid and severe impairment of urine concentrating ability that are reminiscent of MCKD/HNFJ.SIMILARITY: SwissProt: P07911 ## Contains 3 EGF-like domains. & Contains 1 ZP domain. View All» Brand Family: Chemicon View All» Product Name: Anti-Tamm-Horsfall Glycoprotein Antibody | AB733 View All» Antibody Type: Polyclonal Antibody View All» Qty/Pk: 1 mL View All» Format: Serum View All» Host: Sheep View All»
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