A2ML1 Antibody, Biotin conjugated

Code CSB-PA001005LD01HU
Size US$166
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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) A2ML1 Polyclonal antibody
Uniprot No.
Target Names
A2ML1
Alternative Names
A2ML1 antibody; CPAMD9Alpha-2-macroglobulin-like protein 1 antibody; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 9 antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Alpha-2-macroglobulin-like protein 1 protein (625-751AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Biotin
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Tested Applications
ELISA
Protocols
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Is able to inhibit all four classes of proteinases by a unique 'trapping' mechanism. This protein has a peptide stretch, called the 'bait region' which contains specific cleavage sites for different proteinases. When a proteinase cleaves the bait region, a conformational change is induced in the protein which traps the proteinase. The entrapped enzyme remains active against low molecular weight substrates (activity against high molecular weight substrates is greatly reduced). Following cleavage in the bait region a thioester bond is hydrolyzed and mediates the covalent binding of the protein to the proteinase. Displays inhibitory activity against chymotrypsin, papain, thermolysin, subtilisin A and, to a lesser extent, elastase but not trypsin. May play an important role during desquamation by inhibiting extracellular proteases.
Gene References into Functions
  1. The functional assessment supported the pathogenicity of the RAF1 and RIT1 variants of unknown significance (VUSs), while the significance of two VUSs in A2ML1 remained unclear. PMID: 29402968
  2. The indigenous Filipino population has a ~50% prevalence of otitis media. A2ML1 genotype is the primary risk factor for otitis media and main determinant of disease progression, although age, the middle ear microbiome, and social clusters might modulate the effect of the A2ML1 genotype. PMID: 27484237
  3. A2ML1-related otitis media susceptibility may be mediated by changes in the middle ear microbiome. PMID: 27799062
  4. Studies support a role for alpha-2-macroglobulin-like 1 protein (A2ML1) in the pathophysiology of otitis media. PMID: 26121085
  5. our results provide evidence that mutations in A2ML1 are a cause of Noonan-like syndrome, with a variable phenotype ranging from severe to very mild. PMID: 24939586
  6. a new class of target antigens in a paraneoplastic autoimmune multiorgan syndrome PMID: 20805888
  7. alpha2ML1 is the first alpha2macroglobulin family member detected in the epidermis, where it may play an important role during desquamation by inhibiting extracellular proteases. PMID: 16298998
  8. alpha2ML1 binding to the low density lipoprotein receptor-related protein 1 (LRP1) reveals a new role for LRP1 in the human epidermis PMID: 18648652

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Subcellular Location
Secreted.
Protein Families
Protease inhibitor I39 (alpha-2-macroglobulin) family
Tissue Specificity
In the epidermis, expressed predominantly in the granular layer at the apical edge of keratinocytes (at protein level). Also detected in placenta, testis and thymus but not in epithelia of kidney, lung, small intestine or colon.
Database Links

HGNC: 23336

OMIM: 610627

KEGG: hsa:144568

STRING: 9606.ENSP00000299698

UniGene: Hs.620532

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