Human Tamm-Horsfall Glycoprotein

Description
Catalogue Number	AG733
Brand Family	Chemicon®
Trade Name	Chemicon
Description	Tamm-Horsfall Glycoprotein

Description

Catalogue Number

AG733

Brand Family

Chemicon®

Trade Name

Chemicon

Description

Tamm-Horsfall Glycoprotein

Product Information
Presentation	Lyophillized from deionized water containing 0.02% sodium azide.

Product Information

Presentation

Lyophillized from deionized water containing 0.02% sodium azide.

Applications
Key Applications	Positive Control

Applications

Key Applications

Positive Control

Biological Information
Purity	>95%.
Source	Human urine, tested negative for and HIV I/II and HCV antibodies and HBsAg.
Entrez Gene Number	NM_001008389.1 NM_003361.2
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.
Gene Symbol	UMOD THP THGP HNFJ ADMCKD2 uromucoid uromodulin MCKD2 FJHN
UniProt Number	P07911
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.

Biological Information

Purity

>95%.

Source

Human urine, tested negative for and HIV I/II and HCV antibodies and HBsAg.

Entrez Gene Number

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.

Gene Symbol

UMOD
THP
THGP
HNFJ
ADMCKD2
uromucoid
uromodulin
MCKD2
FJHN

UniProt Number

P07911

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.

Product Usage Statements
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.

Product Usage Statements

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.

Storage and Shipping Information
Storage Conditions	Store +2-8°C for up to 3 months after date of receipt. DO NOT FREEZE.

Storage and Shipping Information

Storage Conditions

Store +2-8°C for up to 3 months after date of receipt.

DO NOT FREEZE.

Packaging Information
Material Size	100 µg

Packaging Information

Material Size

100 µg

实验耗材

分子生物学试剂

蛋白与免疫学

生化试剂

细胞及检测试剂

色谱及层析

仪器设备

实验服务

办公用品

Human Tamm-Horsfall Glycoprotein

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