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Journal of Inherited Metabolic Disease

, Volume 39, Issue 2, pp 321–324 | Cite as

Pride and protein

  • William SternEmail author
Garrodian

Abstract

Objective

In Jane Austen’s Pride and Prejudice, members of the Bennet family are either sensible or silly, and males are under-represented. This study searches for an underlying medical diagnosis that explains these features.

Design

Very retrospective literature review.

Participants

Mrs Bennet, her five daughters (Jane, Elizabeth, Mary, Kitty and Lydia), her brother (Mr Gardiner) and her sister (Mrs Phillips).

Main outcome measures

Family tree and associated phenotypes

Methods

The author read Pride and Prejudice. A Bennet family tree was constructed. The number of male and female descendants was analysed using a binomial model. For each character, evidence of behaviour was collected, and members of the Bennet family were categorised as either sensible or silly.

Results

Males are under-represented in Mrs Bennet’s family. Assuming an equal probability of male or female offspring, this reaches statistical significance (binomial model, P = 0.03). Approximately 50 % of females in the family are silly. Silly behaviour is more prevalent during social gatherings.

Conclusions

The family tree suggests an X-linked genetic disorder, fatal in utero or in early life to affected males, explaining the paucity of male offspring. Female carriers survive, but with cognitive difficulties, explaining the approximate 50-50 distribution of sensible and silly females in the family. The exacerbation of silliness during social gatherings may suggest an effect of protein intake, raising suspicions of a disorder of protein metabolism. Ornithine transcarbamylase deficiency is one such condition. Unfortunately, there remain significant challenges in performing genetic testing on fictional characters, so definitive evidence remains elusive. Jane and Elizabeth Bennet do not show signs of the disorder. However, carriers may be asymptomatic; they should be offered genetic counselling before Bingley or Darcy offspring are considered.

Notes

Acknowledgments

The author is grateful to Professor Ley Sander, for starting him off on this journey.

Compliance with ethical standards

Funding

No funding was sought for this study. The author already owned the book, and it is freely available online.

Competing interests

There are no conflicts of interest to declare.

Ethics

No ethical permission has been sought, as the characters involved are fictional.

References

  1. Austen J (1813a) Pride and prejudice, chapter 1Google Scholar
  2. Austen J (1813b) Pride and prejudice, chapter 4Google Scholar
  3. Austen J (1813c) Pride and prejudice, chapter 7Google Scholar
  4. Austen J (1813d) Pride and prejudice, chapter 15Google Scholar
  5. Austen J (1813e) Pride and prejudice, chapter 25Google Scholar
  6. Austen J (1813f) Pride and prejudice, chapter 41Google Scholar
  7. Austen J (1813g) Pride and prejudice, chapter 43Google Scholar
  8. Gordon N (2003) Ornithine transcarbamylase deficiency: a urea cycle defect. Eur J Paediatr Neurol 7(3):115–121CrossRefPubMedGoogle Scholar
  9. Roser M (2015) Child mortality. OurWorldInData.org. http://ourworldindata.org/data/population-growth-vital-statistics/child-mortality/ [Online Resource]

Copyright information

© SSIEM 2015

Authors and Affiliations

  1. 1.Department of Clinical and Experimental EpilepsyNIHR University College London Hospitals Biomedical Research Centre, UCL Institute of NeurologyLondonUK

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