Where is Uromodulin produced?

Uromodulin (also known as Tamm-Horsfall protein) is exclusively produced in the kidney and is the most abundant protein in normal urine.

What does Tamm-Horsfall protein do?

Tamm-Horsfall (TH) glycoprotein is a major protein of normal urine and is the primary component of waxy nephron casts. Tamm-Horsfall protein is of renal origin and has been localized in the thick ascending limb of the loop of Henle and in the distal convoluted tubule.

Where is Tamm-Horsfall protein made?

Tamm-Horsfall protein (THP) is exclusively produced by renal tubular cells of the distal loop of Henle and is the most abundant urinary protein in mammals.

Does uromodulin polymerise?

Uromodulin is the most abundant protein secreted in urine, in which it is found as a high-molecular-weight polymer. Polymerization occurs via its zona pellucida (ZP) domain, a conserved module shared by many extracellular eukaryotic proteins that are able to assemble into matrices.

What is uromodulin kidney disease?

Uromodulin-associated kidney disease is an inherited condition that affects the kidneys. The signs and symptoms of this condition vary, even among members of the same family. Many individuals with uromodulin-associated kidney disease develop high blood levels of a waste product called uric acid.

What is uromodulin associated kidney disease?

Who discovered Tamm Horsfall?

In 1985, Muchmore and Decker [5] identified a 85 kDa glycoprotein in the urine of pregnant women.

Does Uromodulin circulate?

Circulating Uromodulin inhibits systemic oxidative stress by inactivating the TRPM2 channel – PMC.

How do you reverse nephrocalcinosis?

Once nephrocalcinosis is found, it is unlikely to be reversed, however, partial reversal has been reported in patients who have had successful treatment of hypercalciuria and hyperoxaluria following corrective intestinal surgery.

Is Fanconi syndrome fatal?

Symptoms and Signs of Fanconi Syndrome develops, leading to progressive renal failure that may be fatal before adolescence.

What is the most abundant protein in urine?

uromodulin
The most abundant urinary protein, uromodulin (also known as Tamm-Horsfall protein) is structurally related to the zona pellucida proteins and precipitates in the cold (~ 4 °C) and with changes in urine pH and ionic strength [6, 7].

What is Uromodulin kidney disease?

What is hyaline cast in urine?

Casts are clusters of urinary sediment elements (red blood cells, white blood cells, fat bodies, etc.) wrapped in a protein matrix. Cast formation necessarily occurs in the kidney tubules, and the the contents of the casts indicate the nature of the abnormalities occurring in the kidney itself.

What does nephritis feel like?

The symptoms of lupus nephritis may include foamy urine and edema—swelling that occurs when your body has too much fluid, usually in the legs, feet, or ankles, and less often in the hands or face. You may also develop high blood pressure. Check your blood pressure. High blood pressure can be a sign of lupus nephritis.

What does interstitial nephritis feel like?

Chronic tubulointerstitial nephritis. Its symptoms ⁠— itchiness, fatigue, appetite changes, nausea, vomiting, and breathing issues ⁠— can at first look like kidney failure. Usually, urine is excessive, and there are problems breathing.

How do I get rid of calcification on my kidneys?

Loop diuretic medications can help your kidneys move fluid and get rid of extra calcium, especially if you have heart failure. Intravenous bisphosphonates lower blood calcium levels by regulating bone calcium. Dialysis can be performed to rid your blood of extra calcium and waste when you have damaged kidneys.

What is uromodulin?

“Uromodulin: a unique 85-kilodalton immunosuppressive glycoprotein isolated from urine of pregnant women”. Science. 229 (4712): 479–81. Bibcode: 1985Sci…229..479M. doi: 10.1126/science.2409603.

What is the concentration of uromodulin in urine?

While the monomeric molecule has a MW of approximately 85 kDa, it is physiologically present in urine in large aggregates of up to several million Da. When this protein is concentrated at low pH, it forms a gel. Uromodulin represents the most abundant protein in normal human urine (results based on MSMS determinations).

What are the EGF domains of uromodulin?

Uromodulin consists of an N-terminal EGF domain, two calcium-binding EGF domains, a cysteine -rich domain (D8C), a fourth EGF domain and a C-terminal Zona pellucida-like (ZP) domain. The ZP domain polymerizes into filaments, with protruding arms that correspond to the EGF I-III and D8C domains.

What is the difference between mouse and human uromodulin?

Uromodulin forms high molecular weight oligomers that line the kidney tubules. Human Uromodulin is GPI-linked. Human Uromodulin is 78% aa identical to mouse Uromodulin.