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REN gene
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Gene

REN gene

The REN gene provides instructions for making renin, a protein essential for regulating blood pressure and fluid balance in the body.

Normal Function

The REN gene provides instructions for making a protein called renin, which is produced in the kidneys. Renin is part of the renin-angiotensin system, which regulates blood pressure and the balance of fluids and salts. Renin converts angiotensinogen into angiotensin I, which is subsequently converted to angiotensin II. Angiotensin II causes blood vessels to constrict, increasing blood pressure, and stimulates the production of the hormone aldosterone, which triggers the kidneys to absorb water and salt, further increasing blood pressure.

Proper blood pressure during fetal growth is required for the normal development of the kidneys and other tissues. Angiotensin II may also play a direct role in kidney development by affecting growth factors involved in the formation of kidney structures.

Health Conditions Related to Genetic Changes

REN-related kidney disease

At least four mutations in the REN gene cause REN-related kidney disease, a condition leading to kidney failure. Individuals with this condition have one mutated copy and one normal copy of the gene. The mutations occur in the signal sequence of the renin protein, impairing its normal processing. The resulting abnormal protein is toxic to the kidney cells that produce renin; as these cells die off, the renin-angiotensin system is disrupted, causing progressive kidney disease.

Renal tubular dysgenesis

At least 11 mutations in the REN gene cause renal tubular dysgenesis, a severe disorder characterized by abnormal kidney development before birth, the inability to produce urine (anuria), and severe low blood pressure (hypotension). These issues lead to a reduction of amniotic fluid (oligohydramnios) and a set of birth defects known as the Potter sequence.

Most mutations causing this disorder prevent the production of any renin protein, resulting in a nonfunctional renin-angiotensin system. Without this system, the kidneys cannot control blood pressure. Low blood pressure reduces blood flow (hypoperfusion) and oxygen delivery during fetal development, which impairs kidney structure and function.

Other Names for This Gene

  • angiotensin-forming enzyme
  • angiotensinogenase
  • FLJ10761
  • HNFJ2
  • RENI_HUMAN
  • renin precursor, renal
  • renin preproprotein

Frequently Asked Questions

Management focuses on controlling the renin‑angiotensin system with ACE inhibitors or angiotensin‑II receptor blockers, which can reduce intraglomerular pressure and proteinuria. Close monitoring of blood pressure, electrolytes, and kidney labs every 3–6 months is standard. In advanced disease, dialysis or pre‑emptive kidney transplantation may be discussed; transplant outcomes are generally good because the underlying genetic defect does not affect the donor organ.
The most common clues are unexplained progressive decline in kidney function (rising creatinine or reduced eGFR), low‑grade hypertension, and sometimes mild electrolyte abnormalities. In infants, poor weight gain, polyuria, or recurrent urinary tract infections may be the first clues. Prompt referral to a pediatric nephrologist for renal ultrasound and renin‑angiotensin system assessment is advised.
pathogenic REN mutation can cause renal tubular dysgenesis in the fetus, leading to severe hypotension and low amniotic fluid (oligohydramnios). High‑resolution fetal ultrasound and amniotic fluid assessment are recommended each trimester. If abnormalities are seen, a maternal‑fetal medicine specialist may consider early delivery and neonatal intensive care planning. Genetic counseling should be offered to discuss recurrence risk (50 % for each pregnancy).
Genetic testing is the definitive way—usually a targeted REN sequencing panel ordered by a nephrologist or clinical geneticist. If you have a family history of early‑onset kidney disease, ask your doctor for referral to a genetics clinic where they can arrange blood or saliva testing and provide pre‑test counseling.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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