供货周期: | 现货 |
品牌: | 康朗生物 |
规格: | 0.1ml/0.2ml |
货号: | KL6426R |
CAS号: |
Quantity size:
0.2ml
Concentration:
1mg/ml TH糖蛋白抗体 Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
May play a role in regulating the circulating activity of cytokines as it binds to IL-1, IL-2 and TNF with high affinity.
Tissue specificity:Synthesized by kidney. Most abundant protein in normal human urine.
Involvement in disease:Defects in UMOD are the cause of familial juvenile hyperuricemic nephropathy type 1 (HNFJ1) . 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.
Defects in UMOD are the cause of TH糖蛋白抗体 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. his gene encodes uromodulin, the most abundant protein innormal urine. Its excretion in urine follows proteolytic cleavageof the ectodomain of its glycosyl phosphatidylinosital-anchoredcounterpart that is situated on the luminal cell surface of theloop of Henle. Uromodulin may act as a constitutive inhibitor ofcalcium crystallizationTH糖蛋白抗体 in renal fluids. Excretion of uromodulin inurine may provide defense against urinary tract infections causedby uropathogenic bacteria. Defects in this gene are associated withthe autosomal dominant renal disorders medullary cystic kidneydisease-2 (MCKD2) and familial juvenile hyperuricemic nephropathy(FJHN). These disorders are characterized by juvenile onset ofhyperuricemia, gout, and progressive renal failure. While severaltranscript variants may exist for this gene, the full-lengthnatures of only two have been described to date. These tworepresent the major variants of this gene and encode the sameisoform.
TH糖蛋白抗体 Also known as:
ADMCKD2; FJHN; HNFJ; HNFJ1; MCKD2; medullary cystic kidney disease 2 (autosomal dominant); Tamm Horsfall glycoprotein; Tamm Horsfall urinary glycoprotein; Tamm-Horsfall urinary glycoprotein; THGP; THP; Umod; UROM_HUMAN; Uromodulin.
Specificity:
●
Rabbit Polyclonal IgG, affinity purified by Protein A.
●
Reacts with: Human, Mouse, Rat, Pig, Cow, Horse, Sheep, .
●
Immunogen: KLH conjugated synthetic peptide derived from human Uromodulin (405-455aa).
●
TH糖蛋白抗体 Predicted Molecular Weight: 61/64kDa.
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500
Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.
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