A2ML1 Antibody

Code CSB-PA001005LA01HU
Size US$166
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  • Western Blot
    Positive WB detected in: A549 whole cell lysate
    All lanes: A2ML1 antibody at 3.2µg/ml
    Secondary
    Goat polyclonal to rabbit IgG at 1/50000 dilution
    Predicted band size: 162, 106 kDa
    Observed band size: 162 kDa

  • Immunohistochemistry of paraffin-embedded human lung cancer using CSB-PA001005LA01HU at dilution of 1:100

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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
Non-conjugated

The A2ML1 Antibody (Product code: CSB-PA001005LA01HU) is Non-conjugated. For A2ML1 Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA001005LB01HU A2ML1 Antibody, HRP conjugated ELISA
FITC CSB-PA001005LC01HU A2ML1 Antibody, FITC conjugated
Biotin CSB-PA001005LD01HU A2ML1 Antibody, Biotin conjugated ELISA
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, WB, IHC
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:5000
IHC 1:20-1:200
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.
Description

The A2ML1 antibody is generated from the rabbit immunized with a partial recombinant protein corresponding to 625-751 amino acids of human A2ML1 protein. It exhibits the unconjugated IgG isoform. Its specificity for human A2ML1 protein has been validated in ELISA, IHC, and WB applications. The purity of this A2ML1 antibody is greater than 95% using protein G purified.

Its target protein A2ML1 mainly inhibits protease activity, contributing to protecting tissues from degradation by enzymes. A2ML1 also plays a role in several biological processes, including immune response, wound healing, and tissue regeneration. Mutations in the A2ML1 gene have been associated with several developmental disorders, including Noonan syndrome-like disorder with loose anagen hair.

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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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