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- Table of Contents
Facts about Zinc finger protein ZIC 1.
Plays important roles in the early stage of organogenesis of the CNS, as well as through dorsal spinal cord development and maturation of the cerebellum. Involved in the spatial distribution of mossy fiber (MF) neurons within the pontine grey nucleus (PGN).
Human | |
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Gene Name: | ZIC1 |
Uniprot: | Q15915 |
Entrez: | 7545 |
Belongs to: |
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GLI C2H2-type zinc-finger protein family |
Zic family member 1 (odd-paired homolog, Drosophila); ZIC1; ZICZic family member 1 (odd-paired Drosophila homolog); Zinc finger protein 201; Zinc finger protein of the cerebellum 1; ZNF201; ZNF201zinc finger protein ZIC 1
Mass (kDA):
48.309 kDA
Human | |
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Location: | 3q24 |
Sequence: | 3; NC_000003.12 (147409365..147416719) |
CNS. A high level expression is seen in the cerebellum. Detected in the nuclei of the cerebellar granule cell lineage from the progenitor cells of the external germinal layer to the postmigrated cells of the internal granular layer. Detected in medulloblastoma (26/29 cases), but not present in all other tumors examined.
Nucleus. Cytoplasm. Localizes in the cytoplasm in presence of MDFIC overexpression.
The ZIC1 mark is a member in the C2H2 protein family. The ZIC1 marker is especially useful in the detection of medulloblastomas (a type of brain cancer). There are many options for how to use the ZIC1 mark in your research. One option is to conduct an ELISA using a ZIC1/2/3 Cell Based ELISA System Kit.
The ZIC1/2/3 Cell Based ELSA Kit monitors ZIC1/2/3 protein levels in cell cultures. It is a convenient method to test for the effects of activators or treatments on ZIC1/2/3. This kit can be used to screen for drugs and treatments on ZIC1/2/3.
The genome of C2H2-type C2H2 Zinc Finger Proteins contains many homologous units. C2H2 protein finger proteins can be found in worms, flies and humans, for example. The present study extends the findings of previous studies to the second arthropod genome, Daphnia pulex. These results support the hypothesis, that ZIC1 is part of the C2H2-type family of zinc finger proteins.
A novel cDNA coding sequence that encodes Glih2’s zinc fingers region was compared with sequences from the Celera Genomics database. These sequences possess high affinity to the zinc finger area. One sequence encodes for zinc fingers one through two, while the second encodes for zinc fingers 3-5. Further analysis of the mouse cDNA suggests that these two sequences belong to the same gene.
OAZ was duplicated in many vertebrates, including humans. EHZF1, which is closely related to OAZ, has the same function, but its zinc finger clusters vary. The human and mouse orthologs each have 30 zinc fingers, while the Drosophila and Daphnia homologs have more. DmOAZ is a member C2H2-type Zinc Finger Protein Family. It can be found in spermatozoa. Interestingly, despite the similar structure, ZNF521, EHZF, and EHZF both regulate certain genes and are involved BMP signal transduction.
Zic1 belongs the C2HD2-type Zinc Finger Protein family and contains three to four zinc domains. It interacts with another zinc finger protein, MyoD, to play a role in spatial distribution of MF neurons in the pontine gray nucleus. It also regulates axon pathways choice and promotes MF neuromigration to ipsilateral cerebellar territory.
Glih2 is believed to have a similar DNA binding area to Gli proteins. Zic proteins have a lower affinity for Gli DNA-binding sites than Gli protein. This does not necessarily mean that Zic proteins can't bind to DNA. It has been shown that Glih2 zinc-GST fuse protein can bind to DNA, and interact with the promoter.
The poly-ZNFs have been associated with severe abnormalities in brain structure, morphology, and function. These polyZNFs have additional domains called SCAN. They have been associated with abnormal higher-order brain functions. These proteins are found all over the world, in yeasts as well as humans. They have greatly expanded with species development. It is possible that there is a link between C2H2-ZNFs (autism) and this protein.
The fluid-filled fourth cavity in the brain is the most common location for medulloblastomas. The brain is composed of four cavities. The fluid cerebrospinal (CSF), circulates via these channels. Hydrocephalus is a condition in which excess CSF builds up in the brain. It can be caused by tumors in the fourth ventricle. In rare cases, tumors may also involve extraocular muscles.
Medulloblastomas are usually classified as WHO IV tumors. However, the disease can be sub-dissected according to the extent and location of nodularity. For example, tumors with extensive nodularity have the worst prognosis. Recent data suggests that medulloblastomas may be classified into molecular subgroups according to their mutations in specific molecular pathways.
Children are more likely to develop this type of cancer than adults and it can happen at any age. Symptoms of this disease can include seizures or abnormal movements, seizures, or memory loss. There is no cure for medulloblastomas, but chemotherapy is available for treatment. Chemotherapy is given through an IV or central line. The disease can spread to other parts or the brain. It is important to get treatment as soon as possible.
Medulloblastomas usually respond to surgery. The tumor may be removed during surgery or relieved from pressure. Sometimes, complete elimination is not possible. Other treatments include chemotherapy. This can be administered in pill form or through a vein. Some patients may require more aggressive treatment than others. Clinical trials may be an option for them. There are many other options available to patients, including chemotherapy, surgery, stem cell transplant and surgery.
There are several types of medulloblastomas. These are known collectively as group 4 and account in between thirty-five and forty percent of all cases. The likelihood of developing group 4 is three times higher for males than for females. They are also more susceptible to having a few or none of the gene mutations that make them high-risk. If you experience any of these symptoms, a doctor should diagnose you.
Some medulloblastomas are related to chromosomal abnormalities. These changes do NOT occur in childhood. They typically develop at an undetermined developmental stage. These changes can be hereditary. Symptoms vary depending on the cause of the disease, but if a family member has a genetic predisposition, they are more likely to develop the cancer. Medulloblastomas respond well to treatment and are often found early.
Medulloblastoma survival rates can vary widely. Children have a median survival time of two years for medulloblastoma, while adults can live up to seventy per cent. The odds of survival are lower for children under three years. Also, treatments are more limited in younger patients. If you suspect your child has medulloblastoma make sure they get a full diagnosis from their pediatrician.
PMID: 8542595 by Yokota N., et al. Predominant expression of human zic in cerebellar granule cell lineage and medulloblastoma.
PMID: 26340333 by Twigg S.R., et al. Gain-of-function mutations in ZIC1 are associated with coronal craniosynostosis and learning disability.