PCCB Antibody, FITC conjugated
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Description
Key Features of FITC Conjugation
Conjugation Process and Optimization
The conjugation of FITC to PCCB antibodies follows standardized protocols tailored to preserve antigen-binding activity while maximizing fluorescence efficiency.
Protocol Overview
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Buffer Preparation:
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Reaction Conditions:
Critical Factors in Conjugation Efficiency
| Factor | Impact on Conjugation | Source |
|---|---|---|
| pH | FITC reactivity peaks at pH 9.2–9.5 . | |
| Antibody Purity | >95% purity required for optimal labeling . | |
| Sodium Azide Removal | Essential to prevent FITC degradation . |
Applications in Research and Diagnostics
The PCCB antibody, FITC-conjugated, is primarily used to study mitochondrial function, fatty acid metabolism, and diseases like propionic acidemia.
Common Techniques and Dilutions
Impact of FITC Labeling on Antibody Functionality
Studies highlight trade-offs between fluorescence intensity and binding affinity:
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Binding Affinity: Higher FITC labeling indices correlate with reduced antigen-binding capacity .
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Specificity: Over-conjugation increases non-specific staining, necessitating careful optimization .
Comparative Analysis of PCCB Antibodies
| Antibody Catalog No. | Host | Clonality | Conjugate | Target Region | Reactivity |
|---|---|---|---|---|---|
| ABIN7164818 | Rabbit | Polyclonal | FITC | AA 200–314 | Human |
| PA5-102766 | Rabbit | Polyclonal | Unconjugated | Full-length | Human, Mouse |
| 11139-1-AP | Rabbit | Polyclonal | Unconjugated | Full-length | Human, Mouse, Rat |
Key Considerations for Use
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Storage: Store at -20°C in PBS with 50% glycerol to prevent aggregation .
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Titration: Optimal working dilutions vary by application; always validate in-house .
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Cross-Reactivity: Confirm specificity for target species (e.g., human vs. mouse) .
Troubleshooting FITC-Conjugated Antibodies
Product Specs
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Lead Time
Typically, we can ship products within 1-3 business days after receiving your order. Delivery times may vary depending on the purchase method or location. Please consult your local distributors for specific delivery timelines.
Synonyms
DKFZp451E113 antibody; PCCase subunit beta antibody; pccB antibody; PCCB_HUMAN antibody; pccBC Complementation group antibody; Propanoyl CoA:carbon dioxide ligase subunit beta antibody; Propanoyl-CoA:carbon dioxide ligase subunit beta antibody; Propionyl CoA carboxylase beta chain; mitochondrial antibody; propionyl Coenzyme A carboxylase; beta polypeptide antibody; Propionyl-CoA carboxylase ; beta subunit antibody; Propionyl-CoA carboxylase beta chain; mitochondrial antibody; R74805 antibody
Target Names
PCCB
Uniprot No.
Target Background
Function
This antibody targets one of the two subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme crucial for the catabolism of odd-chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine, as well as other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA. Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit subsequently transfers the carboxyl group from carboxylated biotin to propionyl-CoA. Propionyl-CoA carboxylase also exhibits significant activity on butyryl-CoA/butanoyl-CoA, converting it to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA at a considerably lower rate. Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA.
Gene References Into Functions
- Recurrent lactic acid elevations were observed in six out of eight patients. Five of the eight patients received a diagnosis of Autism Spectrum Disorder, and four of them harbored pathogenic variants in the PCCB gene. PMID: 27825584
- The majority of patients exhibited mutations in the PCCA gene (18/25). A total of 26 mutations were identified: 20 in the PCCA gene and 6 in the PCCB gene. Seventeen mutations were novel (14 in PCCA and 3 in PCCB). PMID: 27227689
- The c.-4156_183+3713del mutation represents the first known large deletion impacting the functional integrity of the PCCB gene. PMID: 24863100
- Mutations exert an impact on the homomeric and heteromeric assembly of Propionyl-CoA Carboxylase. PMID: 11749052
- Structure-function analysis of a diverse range of isolated PCCB subunit mutants has illuminated the complex relationship between genotype and phenotype in propionic acidemia. PMID: 12007220
- Research revealed 15 novel PCCB gene mutations and established phenotype-genotype correlations, facilitating the prediction of metabolic outcomes and the implementation of tailored treatments for individual PA patients. PMID: 12559849
- The pathogenicity of R67S, R165Q, and G112D mutations in the PCCB gene in propionic acidemia was investigated. PMID: 12757933
Database Links
Involvement In Disease
Propionic acidemia type II (PA-2)
Protein Families
AccD/PCCB family
Subcellular Location
Mitochondrion matrix.
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