The Wilms gene, also known as the WT1 gene, is a crucial element in kidney development. The spelling of this gene's name uses the International Phonetic Alphabet (IPA) transliteration. The "W" is pronounced as a voiced bilabial fricative "wuh," while the "i" is pronounced as a long vowel sound "eye." The "l" in "Wilms" is pronounced as a lateral approximant "l," and the "m" is pronounced as a bilabial nasal "muh." The consonant cluster "ms" is pronounced as "mss." The word "gene" is pronounced with a voiced velar stop "g" and an "ee" vowel sound.
The Wilms gene refers to a specific gene known as WT1 (Wilms' tumor suppressor gene). The Wilms gene is named after Wilms' tumor, a type of kidney cancer predominantly affecting children. WT1 is a tumor suppressor gene located on chromosome 11p13, and it plays a crucial role in the regulation of normal kidney development during embryogenesis.
This gene is responsible for producing a protein called Wilms' tumor suppressor protein (WT1 protein). The WT1 protein acts as a transcription factor, meaning it controls the expression of various genes involved in the normal development and functioning of the kidneys.
Mutations or abnormal alterations in the Wilms gene can disrupt the normal regulatory functions of the WT1 protein, leading to the development of Wilms' tumor - a type of kidney cancer. In some cases, these mutations can also cause certain genitourinary abnormalities or abnormalities in other organs, such as the gonads.
Scientists and researchers have extensively studied the Wilms gene and its role in kidney development and Wilms' tumor formation. Understanding the molecular mechanisms and functions of this gene has contributed to advancements in diagnoses and treatments for Wilms' tumor, as well as a better understanding of normal kidney development and related disorders.
The term "Wilms gene" is derived from the name Wilms tumor, a type of kidney cancer that primarily affects children. This name honors the German surgeon and pathologist, Max Wilms, who first described this cancer in 1899. Wilms tumor occurs due to specific genetic mutations, and one of the most significant genes associated with this cancer is called WT1 (Wilms tumor 1). Hence, the term "Wilms gene" is used to refer to the WT1 gene and its related functions in the development and progression of Wilms tumor.