Zinc finger protein PLAG1 is a protein that in humans is encoded by the PLAG1 gene. [5] [6]
Zinc finger protein PLAG1 is a protein that in humans is encoded by the PLAG1 gene. [5] [6]
Pleomorphic adenoma gene 1 encodes a zinc finger protein with 2 putative nuclear localization signals. PLAG1, which is developmentally regulated, has been shown to be consistently rearranged in pleomorphic adenomas of the salivary glands. PLAG1 is activated by the reciprocal chromosomal translocations involving 8q12 in a subset of salivary gland pleomorphic adenomas. [6]
PLAG1 has been shown to interact with Karyopherin alpha 2. [7]
An adenoma is a benign tumor of epithelial tissue with glandular origin, glandular characteristics, or both. Adenomas can grow from many glandular organs, including the adrenal glands, pituitary gland, thyroid, prostate, and others. Some adenomas grow from epithelial tissue in nonglandular areas but express glandular tissue structure. Although adenomas are benign, they should be treated as pre-cancerous. Over time adenomas may transform to become malignant, at which point they are called adenocarcinomas. Most adenomas do not transform. However, even though benign, they have the potential to cause serious health complications by compressing other structures and by producing large amounts of hormones in an unregulated, non-feedback-dependent manner. Some adenomas are too small to be seen macroscopically but can still cause clinical symptoms.
Adenoid cystic carcinoma is a rare type of cancer that can exist in many different body sites. This tumor most often occurs in the salivary glands, but it can also be found in many anatomic sites, including the breast, lacrimal gland, lung, brain, Bartholin gland, trachea, and the paranasal sinuses.
Pleomorphic adenoma is a common benign salivary gland neoplasm characterised by neoplastic proliferation of epithelial (ductal) cells along with myoepithelial components, having a malignant potentiality. It is the most common type of salivary gland tumor and the most common tumor of the parotid gland. It derives its name from the architectural Pleomorphism seen by light microscopy. It is also known as "Mixed tumor, salivary gland type", which refers to its dual origin from epithelial and myoepithelial elements as opposed to its pleomorphic appearance.
Canalicular adenoma is a benign, epithelial salivary gland neoplasm arranged in interconnecting cords of columnar cells. This is a very rare benign neoplasm, that makes up about 1% of all salivary gland tumors, or about 4% of all benign salivary gland tumors.
Mucoepidermoid carcinoma (MEC) is the most common type of minor salivary gland malignancy in adults. Mucoepidermoid carcinoma can also be found in other organs, such as bronchi, lacrimal sac, and thyroid gland.
High-mobility group AT-hook 2, also known as HMGA2, is a protein that, in humans, is encoded by the HMGA2 gene.
Importin subunit alpha-1 is a protein that in humans is encoded by the KPNA2 gene.
Zinc finger protein PLAGL1 is a protein that in humans is encoded by the PLAGL1 gene.
CREB-regulated transcription coactivator 1 (CRTC1), previously referred to as TORC1 (Transducer Of Regulated CREB activity 1), is a protein that in humans is encoded by the CRTC1 gene. It is expressed in a limited number of tissues that include fetal brain and liver and adult heart, skeletal muscles, liver and salivary glands and various regions of the adult central nervous system.
Reticulon-1 also known as neuroendocrine-specific protein (NSP) is a protein that in humans is encoded by the RTN1 gene.
Salivary gland tumours, also known as mucous gland adenomas or neoplasms, are tumours that form in the tissues of salivary glands. The salivary glands are classified as major or minor. The major salivary glands consist of the parotid, submandibular, and sublingual glands. The minor salivary glands consist of 800 to 1000 small mucus-secreting glands located throughout the lining of the oral cavity. Patients with these types of tumours may be asymptomatic.
Mastermind-like protein 2 is a protein that in humans is encoded by the MAML2 gene.
Zinc finger protein PLAGL2 is a protein that in humans is encoded by the PLAGL2 gene.
Kallikrein-related peptidase 9 also known as KLK9 is an enzyme which in humans is encoded by the KLK9 gene.
ETV6-NTRK3 gene fusion is the translocation of genetic material between the ETV6 gene located on the short arm of chromosome 12 at position p13.2 and the NTRK3 gene located on the long arm of chromosome 15 at position q25.3 to create the (12;15)(p13;q25) fusion gene, ETV6-NTRK3. This new gene consists of the 5' end of ETV6 fused to the 3' end of NTRK3. ETV6-NTRK3 therefore codes for a chimeric oncoprotein consisting of the helix-loop-helix (HLH) protein dimerization domain of the ETV6 protein fused to the tyrosine kinase domain of the NTRK3 protein. The ETV6 gene codes for the transcription factor protein, ETV6, which suppresses the expression of, and thereby regulates, various genes that in mice are required for normal hematopoiesis as well as the development and maintenance of the vascular network. NTRK3 codes for Tropomyosin receptor kinase C a NT-3 growth factor receptor cell surface protein that when bound to its growth factor ligand, neurotrophin-3, becomes an active tyrosine kinase that phosphorylates tyrosine residues on, and thereby stimulates, signaling proteins that promote the growth, survival, and proliferation of their parent cells. The tyrosine kinase of the ETV6-NTRK3 fusion protein is dysfunctional in that it is continuously active in phosphorylating tyrosine residues on, and thereby continuously stimulating, proteins that promote the growth, survival, and proliferation of their parent cells. In consequence, these cells take on malignant characteristics and are on the pathway of becoming cancerous. Indeed, the ETV6-NTRK3 fusion gene appears to be a critical driver of several types of cancers. It was originally identified in congenital fibrosarcoma and subsequently found in mammary secretory carcinoma, mammary analogue secretory carcinoma of salivary glands, salivary gland–type carcinoma of the thyroid, secretory carcinoma of the skin, congenital fibrosarcoma, congenital mesoblastic nephroma, rare cases of acute myelogenous leukemia, ALK-negative Inflammatory myofibroblastic tumour, cholangiocarcinoma, and radiation-induced papillary thyroid carcinoma.
Salivary duct carcinoma (SDC) is a rare type of aggressive cancer that arises from the salivary glands. It is predominantly seen in men and, generally, has a poor prognosis. Other high grade carcinomas can mimic SDC. About 40-60% of SDC arise in pleomorphic adenomas. Most, if not all, SDCs express androgen receptor by immunohistochemistry. Therapeutically relevant genetic alterations include ERBB2/Her2 amplification, PIK3CA and/or HRAS mutations.
Myoepithelioma of the head and neck, also myoepithelioma, is a salivary gland tumour of the head and neck that is usually benign. When malignant, which is exceedingly rare, they are known as malignant myoepithelioma or Myoepithelial carcinoma, and they account for 1% of the salivary tumors with poor prognosis.
A sialoblastoma is a low-grade salivary gland neoplasm that recapitulates primitive salivary gland anlage. It has previously been referred to as congenital basal cell adenoma, embryoma, or basaloid adenocarcinoma. It is an extremely rare tumor, with less than 100 cases reported worldwide.
Carcinoma ex pleomorphic adenoma is a type of cancer typically found in the parotid gland. It arises from the benign tumour pleomorphic adenoma.
Mammary analogue secretory carcinoma (MASC), also termed MASCSG, is a salivary gland neoplasm. It is a secretory carcinoma which shares the microscopic pathologic features with other types of secretory carcinomas including mammary secretory carcinoma, secretory carcinoma of the skin, and salivary gland–type carcinoma of the thyroid. MASCSG was first described by Skálová et al. in 2010. The authors of this report found a chromosome translocation in certain salivary gland tumors, i.e. a (12;15)(p13;q25) fusion gene mutation. The other secretory carcinoma types carry this fusion gene.
This article incorporates text from the United States National Library of Medicine, which is in the public domain.
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