Pnp

Pro-B

Pro-B

RAG1, RAG2,

ARTEMIS, CERNUNNOS, DNA Ligase 4, DNAPKcs, CD45, CD3

Pre-TCR checkpoint

Pre-BCR checkpoint

Cycling pre-T t

RAG1, RAG2,

ARTEMIS, CERNUNNOS, DNA Ligase 4, DNAPKcs, BTK, 15, Iga

Igp. chain, BLNK, LRRC8

Cycling pre-T t

Large pre-B

FIGURE 20-1 Immunodeficiency caused by defects in B and T cell maturation. Primary immunodeficiencies caused by genetic defects in lymphocyte maturation are shown. These defects may affect T cell maturation alone, B cell maturation alone, or both. CLP, common lymphoid progenitor; DP, double-positive; FoB, follicular B cells; HSC, hematopoietic stem cell; MZB, marginal zone B cells.

FIGURE 20-1 Immunodeficiency caused by defects in B and T cell maturation. Primary immunodeficiencies caused by genetic defects in lymphocyte maturation are shown. These defects may affect T cell maturation alone, B cell maturation alone, or both. CLP, common lymphoid progenitor; DP, double-positive; FoB, follicular B cells; HSC, hematopoietic stem cell; MZB, marginal zone B cells.

have been described in different forms of SCID. About 50% of SCIDs are autosomal recessive; the rest are X-linked. The most common cause of autosomal recessive SCID is deficiency of the enzyme adenosine deaminase, required for purine metabolism. X-linked SCID is caused by mutations in the gene encoding a cytokine receptor component called the common y chain. The individual disorders are described here.

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