Study your flashcards anywhere!

Download the official Cram app for free >

  • Shuffle
    Toggle On
    Toggle Off
  • Alphabetize
    Toggle On
    Toggle Off
  • Front First
    Toggle On
    Toggle Off
  • Both Sides
    Toggle On
    Toggle Off
  • Read
    Toggle On
    Toggle Off
Reading...
Front

How to study your flashcards.

Right/Left arrow keys: Navigate between flashcards.right arrow keyleft arrow key

Up/Down arrow keys: Flip the card between the front and back.down keyup key

H key: Show hint (3rd side).h key

A key: Read text to speech.a key

image

Play button

image

Play button

image

Progress

1/2

Click to flip

2 Cards in this Set

  • Front
  • Back
x-linked disorder -- severity varies w/ age but usually results in fatal infection or lymphoid malignancy

manifests by defective responses to bacterial polysaccharides and by lower-than-average IgM levels

recurrent bacterial infections during aging, gradual loss of humoral and cell-mediated responses

includes thrombocytopenia, eczema

defect is mapped to short arm of X chromosome -- involves cytoskeletal glycoprotein present in lymphoid cells called sialophorin (CD43)
Wiskott-Aldrich Syndrome (WAS)
characterized by extremely low IgG levels and by the absence of other Ig classes

no peripheral B cells and suffer from recurrent bacterial infections

defect in B-cell signal transduction -- due to defect in a transduction molecule called Bruton's tyrosine kinase (Btk)

B cells in these patients remain in the pre-B stage w/ H chains rearranged but L chains in their germ-line configuration
X-linked agammaglobulinemia