Figure 2

Sertoli cell immuno-labelling of vimentine [1 and 2], anti-Müllerian hormone [3], androgen receptor [4] and Leydig cell immuno-labelling of 3β HSD: 3β-hydroxysteroid dehydrogenase [5] in seminiferous tubules of the Hurler syndrome patient [D] and 1.8 [C1 – A], 9 [C2 – B], 12 [C3 – C] and 17 [C4 – E] -year-old controls respectively. Sertoli cells with positive expression of (i) cytoplasmic vimentine or AMH as well as (ii) nucleic AR, are brown stained. For Hurler patient and controls, AR was always expressed in Leydig and myoid cells. AR could be detected in Sertoli cells only at the onset of puberty [C4 and E4] and in our patient [D4]. AMH was strongly expressed for the youngest control [A3], weakly expressed for our Hurler patient [D3] and undetectable for the oldest control [E3]. 3β-HSD was weakly expressed in Leydig cells of the Hurler patient [F3], moderately expressed at the onset of puberty [B5 and C5] and highly expressed in the oldest control [E5]. Scale bar: (1, 3, 4) 50 μm, (2) 30 μm. Footnotes: AMH: Anti-Müllerian Hormone - AR: Androgen Receptor - 3β-HSD: 3β-hydroxysteroid dehydrogenase - C: Control - Es: Elongated spermatid - Sg: Spermatogonia - Sp I: Spermatocyte I - St: Sertoli cell - Vim: Vimentine - y: years.