Immunohistochemistry (IHC)
(The image is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using (ZNF83 Antibody) at dilution 1/50.)
Rabbit anti-Human ZNF83 Polyclonal Antibody | anti-ZNF83 antibody
ZNF83 Antibody
Immunohistochemistry (IHC)
(The image is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using (ZNF83 Antibody) at dilution 1/50.)
Immunohistochemistry (IHC)
(The image is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using (ZNF83 Antibody) at dilution 1/50.)
Immunohistochemistry (IHC)
(The image is immunohistochemistry of paraffin-embedded Human colorectal cancer tissue using (ZNF83 Antibody) at dilution 1/50.)
Immunohistochemistry (IHC)
(The image is immunohistochemistry of paraffin-embedded Human colorectal cancer tissue using (ZNF83 Antibody) at dilution 1/50.)
NCBI and Uniprot Product Information
Uniprot Description
BCS1L: Chaperone necessary for the assembly of mitochondrial respiratory chain complex III. Plays an important role in the maintenance of mitochondrial tubular networks, respiratory chain assembly and formation of the LETM1 complex. Defects in BCS1L are the cause of GRACILE syndrome (GRACILE). GRACILE stands for 'growth retardation, aminoaciduria, cholestasis, iron overload, lactic acidosis, and early death'. It is a recessively inherited lethal disease characterized by fetal growth retardation, lactic acidosis, aminoaciduria, cholestasis, and abnormalities in iron metabolism. Defects in BCS1L are a cause of mitochondrial complex III deficiency (MT-C3D). A disorder of the mitochondrial respiratory chain resulting in a highly variable phenotype depending on which tissues are affected. Clinical features include mitochondrial encephalopathy, psychomotor retardation, ataxia, severe failure to thrive, liver dysfunction, renal tubulopathy, muscle weakness and exercise intolerance. Defects in BCS1L are the cause of Bjoernstad syndrome (BJS). BJS is an autosomal recessive condition characterized by sensorineural hearing loss and pili torti. The hearing loss in BJS is congenital and of variable severity. Pili torti (twisted hairs), a condition in which the hair shafts are flattened at irregular intervals and twisted 180 degrees from the normal axis, making the hair extremely brittle, is usually recognized early in childhood. Belongs to the AAA ATPase family. BCS1 subfamily.
Protein type: Mitochondrial; Membrane protein, integral; Chaperone
Chromosomal Location of Human Ortholog: 2q33
Cellular Component: mitochondrion; mitochondrial respiratory chain complex III
Molecular Function: protein binding; ATP binding
Biological Process: mitochondrial respiratory chain complex I assembly; mitochondrion organization and biogenesis; mitochondrial respiratory chain complex IV assembly
Disease: Leigh Syndrome; Gracile Syndrome; Bjornstad Syndrome; Mitochondrial Complex Iii Deficiency, Nuclear Type 1
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Product Notes
The ZNF83 bcs1l (Catalog #AAA9434660) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The ZNF83 Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's ZNF83 can be used in a range of immunoassay formats including, but not limited to, Immunohistochemistry (IHC). IHC: 1:40-1:200. Researchers should empirically determine the suitability of the ZNF83 bcs1l for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "ZNF83, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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