Monoclonal Antibody Database Contents
Please click on each antibody image for further details.
Advisory: To find all mAbs, use the scroll buttons to descend the lists.
Dystrophin, Utrophin, beta-dystroglycan, alpha-dystroglycan.
Dystrophin is a 427kD cytoskeletal muscle protein containing 3685 amino-acids encoded by 79 exons in the dystrophin gene. Mutations in dystrophin cause Duchenne and Becker muscular dystrophies. Utrophin is a homologous protein found in most tissues. They bind to the C-terminus of beta-dystroglycan in the cell membrane via their WW domains. Alpha-dystroglycan is a glycosylated, extracellular protein which binds to beta-dystroglycan at the sarcolemma. Enquiries about mAb availability to firstname.lastname@example.org Please let me know of any errors or omissions.
The mAb database is followed by a list of dystrophin exon boundaries. Abbreviations: wb = western blot if = immunolocalization ip = immunoprecipitation hu = human mo = mouse xe = xenopus lævis ch = chick dm = drosophila fi = fish (raja clavata) CRX = cross-reacting proteins p = preparation m = mapping a = applications
Huntingtin, Dopamine Receptors and Fukutin.
Huntingtin is a protein of 3144 amino-acids and Mr of 350kDa. The defect in Huntington's disease is due to expansion of a poly-glutamine repeat sequence near the N-terminus, which exerts a deleterious, dominant-negative effect on the survival of specific neurons in the brain.
The D2-type dopamine receptors have been associated, indirectly, with both Parkinson's Disease and schizophrenia. Fukutin is a protein of the Golgi apparatus, where it is involved in the enzymic glycosylation of alpha-dystroglycan. Mutations in fukutin cause the severe congenital disorder, Fukuyama MD.
Spinal Muscular Atrophy - SMN, Gemins 2, 4, 5, 6 & 7, Profilin II.
SMN (Survival of Motor Neurons) is a protein of 294 amino-acids and Mr of 40kDa. Levels of functional protein are greatly reduced in SMA and are related to disease severity. Gemins(2 - 8)form a functiomal complex with SMN. SIP-1 is a protein which interacts directly with SMN and always co-localizes.SIP-1 is also known as Gemin2.
Emery-Dreifuss MD - emerin, lamins, nesprins 1 and 2.
Emerin is a nuclear membrane protein of 254 amino-acids and Mr of 34kDa. It is usually absent or greatly reduced in the X-linked form of EDMD. How its absence causes cardiac conduction defects, wasting of specific muscles and joint contractures is not yet known (reviewed in ref. 34).
Lamin A/C interacts with emerin at the nuclear lamina-membrane interface and LMNA mutations are one of the main causes of autosomal dominant EDMD. Nesprins are giant proteins that project from the outer nuclear membrane into the cytoplasm and are anchored by SUN protein interaction with their C-terminal KASH domains; nesprin mutations can also cause autosomal dominant EDMD.
Myotonic dystrophy - DMPK, Six5, MBNL1-3, MSH3.
DMPK (Myotonic dystrophy protein kinase) is a skeletal and cardiac muscle enzyme of 623 amino-acids and Mr of 85kDa
(larger than predicted Mr of 69kDa). It may be divided into 4 major domains: N-terminal, catalytic
(PK-A type cyclic-AMP-dependent), coiled coil and C-terminal. Myotonic dystrophy is caused by a trinucleotide repeat expansion in the 3'-UTR of DMPK mRNA.
This same repeat also lies close to the 5'-UTR of a second gene, Six5, a member of the Six family of homeobox transcription factors. Although some features of myotonic dystrophy may be influenced by reduced DMPK levels
(muscle wasting, cardiac conduction defects) or reduced Six5 levels (cataracts),most clinical features, including myotonia, appear to be caused by the accumulation of expanded repeat RNA in the nucleus. This repeat accumulation interferes with the normal activity of nuclear factors, such as MBNL or muscleblind. Sequestration of MBNL by expanded repeats is now thought to be the main pathogenic event in myotonic dystrophy. MSH3 is a protein that regulates the size of repeat expansions
and therefore a potential target for therapy.
Creatine kinases mobilize energy rapidly by converting energy stores of creatine phosphate into ATP.
The muscle isoform (M-CK) is particularly important and is partly located at the M-line where it supplies ATP to the actomyosin ATPase for motor activity.
The brain isoform (B-BK) is widely-distributed but M-CK is switched on in striated muscle only as part of a general myogenic gene switch in early development. Creatine kinase is dimeric and the MB-CK dimer is found only in cardiac muscle.
The enzymes are highly-conformational and most antibodies recognize either native or denatured forms, but not both (Webb and Morris, Proteins 41 (2001) 269-278). Serum CK levels are used in the diagnosis of cardiac infarction and inherited muscle disease.
Enquiries about mAb availability to email@example.com Please let me know of any errors or omissions.
Abbreviations: wb = western blot if = immunolocalization ip = immunoprecipitation hu = human mo = mouse xe = xenopus lævis ch = chick dm = drosophila fi = fish (raja clavata) CRX = cross-reacting proteins p = preparation m = mapping a = applications
Zenon: Multiple mAb labelling.
Zenon technology is based on the complexation of primary antibody with dye or enzyme-labeled Fab fragments of second antibodies directed against their Fc regions. The primary antibody types that can be recognized and detect by Zenon labeling reagents are determined by the immunospecificity of the Fab fragments from which they are prepared.
December 2016: We now show antibody concentrations (mouse Ig: micrograms/ml) for many of our most popular mAbs. They vary between 7 and over 400 ug/ml so the data is important for antibody dilution (generally, use 1ug/ml for western blot). You will find the mAb concn after the Clone Number e.g. for MANDYS1 it will be "3B7, 216" showing that the mAb you receive will be 216ug/ml. (These concns are approximate only, as determined by ELISA for mouse IgG).
October 2016: Ten new mAbs against nesprin-1 added, including one specific for the alpha isoform.
November 2015: Species-specificity of nesprin mAb, MANNES1E, has been corrected to rabbit+ but rat-.
May 2015: Isotyping for antibodies against alpha-dystroglycan, beta-dystroglycan, fukutin, dopamine receptors and several dystrophin mAbs added.
December 2014: Antibodies against alpha-dystroglycan (DAG-6F4) and nesprin-2 DV23 domain added.
October 2012: Antibodies against fukutin, the dystroglycanopathy protein, have been added (see under "Huntingtin, dopamine receptors and fukutin")
October 2011: Corrections made to dystrophin exon55-59 mAbs and references are now accessible by clicking "References" at the head of each mAb list.
July 2011: Antibodies against MSH3 protein (myotonic dystrophy) have been added.
February 2010: Antibodies against Nesprin-1 and Nesprin-2 have been added.
October 2008: Antibodies against Creatine Kinase and MBNL1 have been added.
August 2007: Antibodies against 4 Gemins (Gemin4, Gemin5, Gemin6 and Gemin7) and 3 DRD2-like dopamine receptors have been added.
July 2006: Many antibodies now have datasheets available, with western blot and immunolocalization data. Click on the antibody name highlighted in line 1 of the tables to download.