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AB127093

Recombinant Human PCCB protein (His-DHFR)

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Recombinant Human PCCB protein (His-DHFR) is a Human Fragment protein, in the 7 to 266 aa range, expressed in Escherichia coli, with 90%, suitable for SDS-PAGE.

View Alternative Names

PCCase subunit beta, Propanoyl-CoA:carbon dioxide ligase subunit beta, PCCB

Key facts

Purity

90% SDS-PAGE

Expression system

Escherichia coli

Tags

His-DHFR tag

Applications

SDS-PAGE

applications

Biologically active

No

Accession

P05166

Animal free

No

Carrier free

No

Species

Human

Reconstitution

Reconstitute in water

Storage buffer

Constituents: 0.58% Sodium chloride, 0.32% Tris HCl

storage-buffer

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Complementary Products

Primary Antibodies

AB187686

Anti-PCCA antibody - C-terminal

Immunohistochemistry (Frozen sections) - Anti-PCCA antibody - C-terminal (AB187686)

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Proteins & Peptides

AB159059

Recombinant Human PCCA protein (GST tag N-Terminus)

SDS-PAGE - Recombinant Human PCCA protein (AB159059)

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Primary Antibodies

AB96729

Anti-PCCB antibody

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-PCCB antibody (AB96729)

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Proteins & Peptides

AB177265

Recombinant human MAP4K3/GLK protein (Tagged)

Functional Studies - Recombinant human MAP4K3/GLK protein (Tagged) (AB177265)

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "SDS-PAGE": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
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Sequence info

[{"sequence":"","proteinLength":"Fragment","predictedMolecularWeight":"27.57 kDa","actualMolecularWeight":null,"aminoAcidEnd":266,"aminoAcidStart":7,"nature":"Recombinant","expressionSystem":null,"accessionNumber":"P05166","tags":[{"tag":"His-DHFR","terminus":""}]}]
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Properties and storage information

Shipped at conditions
Blue Ice
Appropriate short-term storage conditions
-20°C
Appropriate long-term storage conditions
-20°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle
False
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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

PCCB also known as Propionyl-CoA Carboxylase β subunit is an enzyme component that plays an important role in metabolizing certain amino acids and odd-chain fatty acids. This protein partners with the alpha subunit to form a dimeric enzyme with a total mass of approximately 750 kDa. PCCB is mainly expressed in the liver but its expression also occurs in other tissues such as the kidney and heart where energy and metabolism play key roles.
Biological function summary

Propionyl-CoA Carboxylase β subunit takes part in the process of carboxylation of propionyl-CoA to methylmalonyl-CoA an essential step in catabolism. This subunit contributes to the function of the complex necessary for efficient energy production and proper detoxification of specific intermediary products. In this enzymatic action it works jointly with the alpha subunit that harbors the biotin-binding sites important for transferring carbon dioxide.

Pathways

PCCB is an important component in the propionic acid pathway and is critical for maintaining the balance in mitochondrial fatty acid synthesis. Its function plays a central role in the catabolism of certain amino acids including valine isoleucine and methionine as well as odd-chain fatty acids. Propionyl-CoA Carboxylase β subunit’s action works closely with methylmalonyl-CoA mutase assisting in metabolic conversion that takes place after the initial carboxylation step.

Deficiencies or mutations in PCCB are linked to propionic acidemia a metabolic disorder that interrupts normal propionic acid breakdown leading to an accumulation that is toxic to the body. This deficiency relates it to methylmalonic acidemia if methylmalonyl-CoA mutase is affected often causing similar clinical symptoms such as developmental delays vomiting and metabolic crises. Understanding the function and pathways of PCCB helps to develop targeted therapies that address its metabolic roles and its involvement in these disorders.
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Specifications

Form

Lyophilized

Additional notes

Purified via His tag

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General info

Function

This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites (PubMed : 15890657, PubMed : 6765947). Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA (PubMed : 15890657, PubMed : 6765947). 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 then transfers the carboxyl group from carboxylated biotin to propionyl-CoA (By similarity). Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA at a much lower rate (PubMed : 6765947). Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA (By similarity).

Sequence similarities

Belongs to the AccD/PCCB family.

Subcellular localisation

Mitochondrion matrix

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Product protocols

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Target data

This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites (PubMed : 15890657, PubMed : 6765947). Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA (PubMed : 15890657, PubMed : 6765947). 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 then transfers the carboxyl group from carboxylated biotin to propionyl-CoA (By similarity). Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA at a much lower rate (PubMed : 6765947). Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA (By similarity).
See full target information PCCB
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