UPSC MainsZOOLOGY-PAPER-I202210 Marks150 Words
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Q19.

Klinefelter's syndrome and Turner's syndrome in humans.

How to Approach

This question requires a comparative discussion of two chromosomal disorders – Klinefelter’s syndrome and Turner’s syndrome. The answer should focus on the chromosomal abnormality causing each syndrome, their respective symptoms (physical and developmental), and the underlying genetic mechanisms. A structured approach comparing the two syndromes using a table would be beneficial. Focus on human genetics and medical implications. Avoid overly technical jargon and maintain clarity.

Model Answer

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Introduction

Human chromosomal disorders arise due to abnormalities in chromosome number or structure. Klinefelter’s syndrome and Turner’s syndrome are two well-known examples of sex chromosome aneuploidies, impacting sexual development and overall health. These conditions, though relatively rare, provide valuable insights into the role of sex chromosomes in human development and highlight the consequences of genetic imbalances. Understanding these syndromes is crucial in the field of medical genetics and reproductive health, allowing for early diagnosis and potential interventions.

Klinefelter’s Syndrome (XXY)

Klinefelter’s syndrome affects males and is characterized by the presence of an extra X chromosome (XXY). This chromosomal abnormality occurs due to non-disjunction during meiosis, either in the mother’s egg or the father’s sperm.

  • Symptoms: Individuals with Klinefelter’s syndrome often exhibit small testes, reduced facial and body hair, gynecomastia (breast enlargement), infertility, and learning disabilities. They may also be taller than average with longer legs.
  • Genetic Mechanism: The presence of the extra X chromosome disrupts normal sexual development, leading to reduced testosterone production.
  • Diagnosis: Typically diagnosed during puberty or through genetic testing (karyotyping).

Turner’s Syndrome (XO)

Turner’s syndrome affects females and is characterized by the complete or partial absence of one X chromosome (XO). This can occur due to non-disjunction during meiosis or through post-zygotic loss of the X chromosome.

  • Symptoms: Individuals with Turner’s syndrome often exhibit short stature, ovarian failure (leading to infertility), a webbed neck, a broad chest with widely spaced nipples, and heart defects. They may also have learning difficulties.
  • Genetic Mechanism: The absence of one X chromosome disrupts normal ovarian development and leads to a range of physical and developmental abnormalities.
  • Diagnosis: Often diagnosed during infancy or childhood due to short stature or delayed puberty, confirmed through karyotyping.

Comparative Analysis

Feature Klinefelter’s Syndrome (XXY) Turner’s Syndrome (XO)
Affected Sex Males Females
Chromosomal Abnormality XXY XO
Primary Symptoms Small testes, gynecomastia, infertility, reduced body hair Short stature, ovarian failure, webbed neck, heart defects
Hormonal Imbalance Low testosterone Estrogen deficiency
Cognitive Effects Learning disabilities Learning difficulties
Fertility Infertility Infertility

Both syndromes are not typically inherited but arise from random chromosomal errors during gamete formation. While there is no cure for either syndrome, hormone therapy (testosterone for Klinefelter’s syndrome and estrogen for Turner’s syndrome) can help manage some of the symptoms and improve quality of life. Early diagnosis and intervention are crucial for optimizing developmental outcomes.

Conclusion

Klinefelter’s and Turner’s syndromes represent significant examples of sex chromosome aneuploidies, each presenting unique challenges to affected individuals. Understanding the underlying genetic mechanisms and associated phenotypic expressions is vital for accurate diagnosis, appropriate management, and genetic counseling. Continued research into the molecular basis of these syndromes may lead to novel therapeutic strategies aimed at mitigating their effects and improving the lives of those affected.

Answer Length

This is a comprehensive model answer for learning purposes and may exceed the word limit. In the exam, always adhere to the prescribed word count.

Additional Resources

Key Definitions

Aneuploidy
Aneuploidy refers to an abnormal number of chromosomes in a cell, differing from the normal diploid number (2n). It can involve the loss (monosomy) or gain (trisomy) of one or more chromosomes.
Non-disjunction
Non-disjunction is the failure of homologous chromosomes or sister chromatids to separate properly during cell division (meiosis or mitosis), leading to an abnormal number of chromosomes in the daughter cells.

Key Statistics

Klinefelter syndrome occurs in approximately 1 in 500 to 1,000 male births.

Source: National Institutes of Health (NIH), 2023 (Knowledge Cutoff)

Turner syndrome affects approximately 1 in 2,000 to 2,500 female births.

Source: Mayo Clinic, 2024 (Knowledge Cutoff)

Examples

Fertility Treatment and Klinefelter's Syndrome

While Klinefelter's syndrome typically causes infertility, advancements in assisted reproductive technologies (ART), such as testicular sperm extraction (TESE) combined with intracytoplasmic sperm injection (ICSI), have allowed some men with Klinefelter's syndrome to father children.

Frequently Asked Questions

Can individuals with Turner's syndrome lead normal lives?

With appropriate medical management, including hormone therapy and monitoring for associated health conditions like heart defects, individuals with Turner's syndrome can lead fulfilling and productive lives. However, they will likely require lifelong medical care.

Topics Covered

GeneticsMedicineBiologyChromosomal DisordersGenetic AbnormalitiesHuman Genetics