Male infertility with spermatogenesis disorder due to single gene mutation

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Overview

Male infertility with spermatogenesis disorder due to single gene mutation is a group of rare genetic conditions in which a change (mutation) in a single gene disrupts the normal production of sperm in the testes. Spermatogenesis is the process by which the body makes mature sperm cells. When a key gene involved in this process is not working properly, it can lead to very low sperm counts (oligozoospermia), no sperm in the semen at all (azoospermia), or sperm that are abnormally shaped or unable to move well. Men with this condition typically appear healthy otherwise and usually discover the problem when they have difficulty conceiving a child with their partner. There are many different genes that can cause this condition, and the specific gene involved determines the exact pattern of inheritance and severity. Some of the most commonly implicated genes include TEX11, SYCP3, NR5A1, DMRT1, and many others. Because so many genes can be responsible, this is considered a genetically heterogeneous group of disorders rather than a single disease. Treatment options are currently limited. Assisted reproductive technologies such as intracytoplasmic sperm injection (ICSI) may help some men father biological children if any viable sperm can be retrieved, sometimes through testicular sperm extraction (TESE). Genetic counseling is strongly recommended to understand the risk of passing the condition to future children. Research into gene therapy and other targeted treatments is ongoing but not yet available for clinical use.

Key symptoms:

Inability to conceive a child after one year of tryingVery low sperm countNo sperm found in the semenAbnormally shaped spermSperm that cannot swim properlySmall or underdeveloped testes in some casesNormal sexual function and libido in most casesNormal male physical appearance in most casesPossible history of undescended testes in childhood

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

1 available

VIZIMPRO

dacomitinib· Pfizer, Inc.
VIZIMPRO is indicated for the first-line treatment of patients with metastatic non-small cell lung cancer (NSCLC) with epidermal growth factor receptor (EGFR) exon 19 deletion or exon 21 L858R substit

VIZIMPRO is indicated for the first-line treatment of patients with metastatic non-small cell lung cancer (NSCLC) with epidermal growth factor receptor (EGFR) exon 19 deletion or exon 21 L858R substitution mutations as detected by an FDA-approved test

No actively recruiting trials found for Male infertility with spermatogenesis disorder due to single gene mutation at this time.

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No specialists are currently listed for Male infertility with spermatogenesis disorder due to single gene mutation.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

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Community

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Caregiver Resources

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.Which specific gene mutation is causing my infertility, and what does it mean for my chances of having biological children?,Is there any chance of finding sperm through surgical retrieval like micro-TESE?,What are my options for assisted reproduction, and what are the success rates?,If I use my own sperm for IVF/ICSI, could my sons inherit this condition?,Should my family members be tested for this gene mutation?,Are there any clinical trials or new treatments being studied for my specific condition?,Can you recommend a genetic counselor who specializes in reproductive genetics?

Common questions about Male infertility with spermatogenesis disorder due to single gene mutation

What is Male infertility with spermatogenesis disorder due to single gene mutation?

Male infertility with spermatogenesis disorder due to single gene mutation is a group of rare genetic conditions in which a change (mutation) in a single gene disrupts the normal production of sperm in the testes. Spermatogenesis is the process by which the body makes mature sperm cells. When a key gene involved in this process is not working properly, it can lead to very low sperm counts (oligozoospermia), no sperm in the semen at all (azoospermia), or sperm that are abnormally shaped or unable to move well. Men with this condition typically appear healthy otherwise and usually discover the p

At what age does Male infertility with spermatogenesis disorder due to single gene mutation typically begin?

Typical onset of Male infertility with spermatogenesis disorder due to single gene mutation is adult. Age of onset can vary across affected individuals.