Mouse Alpha-1-antitrypsin Monoclonal Antibody | anti-SERPINA1 antibody

Alpha-1-antitrypsin, Human, mAb 2C1

Cat. Num. AAA14611

• Gene Names: SERPINA1; PI; A1A; AAT; PI1; A1AT; PRO2275; alpha1AT
• Applications: Immunohistochemistry, Immunofluorescence, Immunoprecipitation, Western Blot
• Purity: Protein G
• Synonyms: Alpha-1-antitrypsin, Antibody; Alpha-1-antitrypsin, Human, mAb 2C1; Alpha-1 protease inhibitor, Alpha-1-antiproteinase, Serpin A1, A1AT Gene name: SERPINA1; anti-SERPINA1 antibody

• Host: Mouse
• Clonality: Monoclonal
• Isotype: IgG1
• Clone Number: 2C1
• Purity/Purification: Protein G
• Form/Format: 0.2 um filtered in PBS+0.1%BSA+0.02%NaN3
• Concentration: 100 ug/ml (varies by lot)
• Sequence Length: 418

• Applicable Applications for anti-SERPINA1 antibody: Immunohistochemistry - Paraffin (IHC-P), Immunofluorescence (IF), Immunoassays (IA), Immunoprecipitation (IP), Western Blot (WB)
• Application Notes: Dilutions to be used depend on detection system applied. It is recommended that users test the reagent and determine their own optimal dilutions. The typical starting working dilution is 1:50.; WB: A non-denaturating gel was used. The bands obtained were ~110, 165, 220 and 275 kDa.; IA: AAA14611 can be used as capture and detection antibody.; Positive control: Human tonsil and kidney; Negative control: For Western blot, any irrelevant protein can be used.
• Immunogen: Z a1-antitrypsin polymers prepared from a1-antitrypsin purified from the plasma of PI*Z homozygotes
• Preparation and Storage: Product should be stored at 4°C. Under recommended storage conditions, product is stable for one year.

IHC (Immunohistochemistry)

(IHC: sections of human tonsil . (AAA14611) was used in a concentration of 2 ug/mL.)

IHC (Immunohistochemistry)

(IHC: sections of human kidney: (AAA14611) was used in a concentratoin of 2 ug/mL)

WB (Western Blot)

(Western Blot with Za1AT polymer lysate (20ug) and (AAA14611) in 1,2 and 4 ug/mL (respectively lane 1,2, and 3).)

Application Data

(Application Notes)

Related Product Information for anti-SERPINA1 antibody

The monoclonal antibody clone 2C1 recognizes polymeric forms of human alpha-1-antitrypsin. Alpha-1-antitrypsin is the most abundant circulating protease inhibitor. Serpinopathies are conformational diseases characterized by the polymerization and intracellular retention of members of the serine protease inhibitor or serpin superfamily of proteins.1 The best known is a1-antitrypsin deficiency, with the most common severe deficiency allele being the Z mutation (Glu342Lys). The severe Z deficiency allele (Glu342Lys) causes the protein to undergo a conformational transition and form ordered polymers that are retained within hepatocytes. This causes neonatal hepatitis, cirrhosis, and hepatocellular carcinoma.Clone 2C1 recognizes polymers formed by Z alpha1-antitrypsin in vivo. It also recognizes polymers formed by the Siiyama (Ser53Phe) and Brescia (Gly225Arg) mutants, and the novel His334Asp shutter domain mutant of a1-antitrypsin that is associated with prolonged neonatal jaundice in a 6-week-old boy. These data show that Z and shutter domain mutants form polymers with a shared epitope.

References

1. Miranda, E et al; A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with alpha1-antitrypsin deficiency. Hepatology 2010, 52: 1078. 2. Ekeowa, U et al; Defining the mechanism of polymerization in the serpinopathies. PNAS 2010, 107: 17146. 3. Morris, H et al; ANCA-associated vasculitis is linked to carriage of the Z allele of alpha1 antitrypsin and its polymers. Ann Rheum Dis 2011, 70: 1851. 4. Yamasaki, M et al; Molecular basis of alpha1-antitrypsin deficiency revealed by the structure of a domain-swapped trimer. EMBO reports 2011, 12: 1011.

NCBI and Uniprot Product Information

• NCBI GI #: 189163542
• NCBI GeneID: 5265
• NCBI Accession #: NP_001121179.1
• NCBI GenBank Nucleotide #: NM_001127707.1
• UniProt Accession #: P01009; Q0PVP5; Q13672; Q53XB8; Q5U0M1; Q7M4R2; Q86U18; Q86U19; Q96BF9; Q96ES1; A6PX14; B2RDQ8
• NCBI Official Full Name: alpha-1-antitrypsin
• NCBI Official Synonym Full Names: serpin family A member 1
• NCBI Official Symbol: SERPINA1
• NCBI Official Synonym Symbols: PI; A1A; AAT; PI1; A1AT; PRO2275; alpha1AT
• NCBI Protein Information: alpha-1-antitrypsin
• UniProt Protein Name: Alpha-1-antitrypsin
• UniProt Gene Name: SERPINA1
• UniProt Synonym Gene Names: AAT; PI; SPAAT