antibody

Sheep anti Uromucoid

MBS316005

1 ml

375 USD

polyclonal

sheep

nonconjugated

Antibody

Sheep anti Uromucoid

Uromucoid (Tamm Horsefall Protein)

Sheep Antibody to Human Uromucoid

uromodulin; Uromodulin; uromodulin; uromucoid; OTTHUMP00000162212; OTTHUMP00000162213; Tamm-Horsfall glycoprotein; tamm-Horsfall urinary glycoprotein; uromodulin (uromucoid, Tamm-Horsfall glycoprotein); uromodulin; Tamm-Horsfall urinary glycoprotein

UMOD; UMOD; THP; FJHN; HNFJ; THGP; HNFJ1; MCKD2; ADMCKD2

UROM_HUMAN

Polyclonal

Sheep

640

Uromucoid (Tamm Horsefall Protein). Gives a single arc when tested by IEP against fresh urine. No arcs are visible when tested by IEP against serum. Identity has been confirmed by double diffusion (Ouchterlony) against uromucoid and a known anti-uromuco

Adsorbed to monospecificity by use of solid-phase adsorbants followed by ion exchange chromatography. The titer is adjusted so that inter-batch variation is within 10%. Product is 0.2um filtered.

Purified, Liquid

14.8mg/ml (OD280nm E^1% = 14.5)

Upon receipt, store at 2 to 8 degree C. Slight precipitation can occur on storage, which may be removed by centrifugation, and should not affect performance characteristics.

Immunogen: Human Uromucoid, purified from human urine

Buffer: Glycine buffered saline, pH 7.4. Preservative: 0.099% Sodium azide, 0.1% E-Amino-n-Caproic acid, 0.01% Benzamidine, 1mM EDTA. Important Note: Centrifuge before opening to ensure complete recovery of vial contents. Warnings: This product contains sodium azide, which has been classified as Xn (Harmful), in European Directive 67/548/EEC in the concentration range of 0.1-1.0%. When disposing of this reagent through lead or copper plumbing, flush with copious volumes of water to prevent azide build-up in drains.

Polyclonal Antibodies to Proteins and Bioactive Peptides

Stricklett, P.K., et al., (2003), "Thick ascending limb-specific expression of Cre recombinase", Am. J. Physiol. Renal. Physiol., 285: F33-F39

59850812

NP_003352.2

NM_003361.2

P07911

69,761 Da

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. [provided by RefSeq]

Function: Uromodulin: Functions in biogenesis and organization of the apical membrane of epithelial cells of the thick ascending limb of Henle's loop (TALH), where it promotes formation of complex filamentous gel-like structure providing the water barrier permeability. May serves as a receptor for binding and endocytosis for cytokines (IL-1, IL-2) and TNF. Facilates neutrophil migration across renal epithelial. Ref.10Uromodulin, secreted form: Secreted into urine after proteolytically cleaveage. Into the urine, may contribute to colloid osmotic pressure, retards passage of positively charged electrolytes, prevents urinary tract infection and modulates formation of supersaturated salts and their crystals. Ref.10. Subcellular location: Apical cell membrane; Lipid-anchor GPI-anchor. Basolateral cell membrane; Lipid-anchor GPI-anchor. Cell projection cilium membrane. Note: Only a small fraction is sort to the basolateral pole of tubular epithelial cells compared to apical localization. Secreted into urine after cleavage. Colocalized with NPHP1 and KIF3A. Ref.8 Ref.9 Ref.10 Ref.11Uromodulin, secreted form: Secreted Ref.8 Ref.10 Ref.11. Tissue specificity: Synthesized exclusively in the kidney. Expressed exclusively by epithelial cells of the thick ascending limb of Henle's loop (TALH) and of distal convoluted tubule lumen. Most abundant protein in normal urine. Ref.8. Domain: The ZP domain is involved in the polymerization of the protein to promote the formation of complex gel-like structure. Post-translational modification: N-glycosylated. By similarity.Proteolytically cleaved at a conserved C-terminal proteolytic cleavage site to generate the secreted form found into urine. Ref.7. Involvement in disease: Defects in UMOD are the cause of familial juvenile hyperuricemic nephropathy type 1 (HNFJ1) [. MIM:162000]. HNFJ1 is a renal disease characterized by juvenil onset of hyperuricemia, polyuria, progressive renal failure, and gout. The disease is associated with interstitial pathological changes resulting in fibrosis. Ref.3 Ref.12 Ref.14Defects in UMOD are the cause of medullary cystic kidney disease type 2 (MCKD2) [. MIM:603860]. MCKD2 is a form of tubulointerstitial nephropathy characterized by formation of renal cysts at the corticomedullary junction. It is characterized by adult onset of impaired renal function and salt wasting resulting in end-stage renal failure by the sixth decade. Ref.3 Ref.13 Ref.15Defects in UMOD are the cause of glomerulocystic kidney disease with hyperuricemia and isosthenuria (GCKDHI) [. MIM:609886]. GCKDHI is a renal disorder characterized by a cystic dilation of Bowman space, a collapse of glomerular tuft, and hyperuricemia due to low fractional excretion of uric acid and severe impairment of urine concentrating ability. Ref.13. Miscellaneous: A specific, but as yet unidentified, protease(s) cleaves off and releases UMOD into urine. Sequence similarities: Contains 3 EGF-like domains.Contains 1 ZP domain.

1 ml

375 USD