It is a zinc finger gene on the Y chromosome known as ZFY.
It was genetically mapped by the study of patients with Klinefelter and Turner syndromes.
The expressed SRY protein has a characteristic highmobility group (HMG), DNA-binding domain.
It is synonymous with Sry in humans.
It is synonymous with the H-Y antigen.
It is secreted by the fetal Leydig cells.
It is secreted by the fetal testis at 10 weeks of gestation, after testosterone production has begun.
It acts systemically to produce müllerian regression.
It is secreted at 7 to 8 weeks of gestation, representing the initial endocrine function of the fetal testis.
It functions normally in patients with hernia uteri inguinale.
The gene was originally isolated in cloning experiments and localized to the X chromosome.
Loss of WT1 function has not been associated with genitourinary anomalies.
Duplication of WT1 has been associated with dosagesensitive sex reversal.
Mutations in WT1 can result in either Denys-Drash or Frasier syndrome.
Mutations in WT1 are associated with adrenocortical carcinoma.
It enters target tissue by active diffusion.
It results in regression of the müllerian ducts.
It acts locally to virilize the urogenital sinus and genital tubercle.
It acts locally to virilize the internal wolffian duct structures.
It is produced primarily by the adrenal gland.
It is secreted in large quantities by the fetal testis.
It produces virilization of the urogenital sinus.
It produces virilization of wolffian duct structures.
It is converted by 5α-reductase to testosterone in target tissues.
It acts locally to produce regression of müllerian structures.
They are at increased risk for development of adenocarcinoma of the breast.
They are characteristically fertile.
They have at least one X and two Y chromosomes.
They undergo replacement of Leydig cells with hyaline.
They bear little resemblance to XX males.
in the presence of a Y chromosome results in risk for development of gonadoblastoma.
can descend to the scrotum.
in the presence of a Y chromosome results in increased risk for development of seminoma.
is located in the round ligament.
has a reduced number of oocytes.
They lack the somatic defects associated with Turner syndrome.
They are at lesser risk for gonadal tumors than are patients with Turner syndrome.
They derive similar benefit from synthetic growth hormone as do patients with Turner syndrome.
They have gonadal histology different from that of patients with Turner syndrome.
They frequently have chromosomal anomalies.
Nephropathy with early-onset proteinuria
Progressive renal failure
They have normal testosterone and elevated estradiol levels.
They have castrate testosterone and elevated gonadotropin levels.
They have castrate testosterone and normal gonadotropin levels.
They have normal testosterone but decreased DHT levels.
They have normal follicle-stimulating hormone but decreased luteinizing hormone levels.
It is found in the minority of patients.
It is impossible to cleave surgically.
It can be unilateral or bilateral.
It cannot descend from the retroperitoneum.
It has testicular and ovarian elements randomly distributed.
the impossibility of precisely dividing an ovotestis surgically.
the familial pattern of inheritance of the disorder.
the unresponsiveness of the external genitalia to testosterone.
that malignant degeneration of gonads does not occur.
the potential for fertility.
It occurs as a result of gene inactivation in the majority of cases.
It is transmitted in an autosomal dominant pattern.
It occurs with simple virilization in 75% of cases and salt wasting in 25% of cases.
It accounts for 99% of CAH cases.
It occurs with a predictable phenotype.
is demonstrated to be effective.
acts by suppressing maternal corticotropin.
is initiated after a diagnosis of CAH is confirmed.
is of no risk to the fetus.
is appropriate therapy in seven of eight at-risk fetuses.
They are transmitted in an autosomal dominant pattern.
They may be associated with fertility.
They appear clinically with a predictable phenotype.
They may involve impaired glucocorticoid and mineralocorticoid synthesis.
They are associated with persistent müllerian structures.
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