Figure 4
From: Metabolite and thymocyte development defects in ADA-SCID mice receiving enzyme replacement therapy
Thymus cellularity is not fully restored in PEG-ADA-treated mice. (A) In vivo images of adult thymi taken from control (Ada+/+) and 3-month-PEG-ADA-treated (Ada−/−) mice. (B) H&E staining of paraffin-embedded thymus sections from control (Ada+/+) and 3-month-PEG-ADA-treated (Ada−/−) mice. C = cortex, heavily filled with developing immature thymocytes; M = medulla, contains a mixture of helper (CD4 +) and cytotoxic (CD8 +) T cells. (C and D) FACS analysis of unfractionated thymocytes gated for: DN (CD4−, CD8−), DP (CD4+, CD8+), CD4-SP (CD4+, CD8−) and CD8-SP (CD4−, CD8+) populations (C), and Linneg thymocytes gated for: DN1 (CD44+, CD25−) DN2 (CD44+, CD25+) DN3 (CD44−, CD25+) DN4 (CD44−, CD25−) populations (D). All thymocytes were from control (Ada+/+) and 3-month-PEG-ADA-treated (Ada−/−) mice. (E) Bar graph representing weight of thymi from control (Ada+/+ and Ada+/−) and 3-month-PEG-ADA-treated (Ada−/−) mice (n = 5, 7, 11). (F and G) Bar graphs representing absolute cell numbers of populations shown in (C) and (D) (n = 6, 7). *P < 0.05, **P < 0.01 and ***P < 0.001. NS = statistically not significant.
