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aldehyde dehydrogenase 4 family, member A1, Polyclonal Antibody

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产品名称: aldehyde dehydrogenase 4 family, member A1, Polyclonal Antibody
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简单介绍

aldehyde dehydrogenase 4 family, member A1, Polyclonal Antibody


aldehyde dehydrogenase 4 family, member A1, Polyclonal Antibody  的详细介绍
Product Name

aldehyde dehydrogenase 4 family, member A1 (ALDH4A1), Polyclonal Antibody

Full Product Name

Rabbit anti-human aldehyde dehydrogenase 4 family, member A1 polyclonal Antibody

Product Synonym Names
aldehyde dehydrogenase 4 family; member A1; ALDH4A1; ALDH4; P5CD; P5CDh; P5CDhL; P5CDhS
Product Gene Name

anti-ALDH4A1 antibody

[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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OMIM
606811
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human, Mouse, Rat
Purity/Purification
Antigen Affinity Purified
Immunogen
Human ALDH4A1
Storage Buffer
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20 degree C, Avoid freeze / thaw cycles.
Santa Cruz Alternative
Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-69145 / sc-130948
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of anti-ALDH4A1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Applications Tested/Suitable for anti-ALDH4A1 antibody
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
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NCBI/Uniprot data below describe general gene information for ALDH4A1. It may not necessarily be applicable to this product.
NCBI GI #
23271000
NCBI GeneID
8659
NCBI Accession #
AAH23600.1 [Other Products]
UniProt Secondary Accession #
Q16882; Q53HU4; Q5JNV6; Q8IZ38; Q96IF0; Q9UDI6; A8K1Q7; B4DGE4; D2D4A3[Other Products]
UniProt Related Accession #
P30038[Other Products]
Molecular Weight
56,043 Da[Similar Products]
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NCBI Official Full Name
Aldehyde dehydrogenase 4 family, member A1
NCBI Official Synonym Full Names
aldehyde dehydrogenase 4 family, member A1
NCBI Official Symbol
ALDH4A1  [Similar Products]
NCBI Official Synonym Symbols
P5CD; ALDH4; P5CDh
  [Similar Products]
NCBI Protein Information
delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial; P5C dehydrogenase; aldehyde dehydrogenase family 4 member A1; L-glutamate gamma-semialdehyde dehydrogenase; mitochondrial delta-1-pyrroline 5-carboxylate dehydrogenase
UniProt Protein Name
Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
UniProt Synonym Protein Names
Aldehyde dehydrogenase family 4 member A1; L-glutamate gamma-semialdehyde dehydrogenase
UniProt Gene Name
ALDH4A1  [Similar Products]
UniProt Synonym Gene Names
ALDH4; P5CDH; P5C dehydrogenase  [Similar Products]
UniProt Entry Name
AL4A1_HUMAN
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NCBI Summary for ALDH4A1
This protein belongs to the aldehyde dehydrogenase family of proteins. This enzyme is a mitochondrial matrix NAD-dependent dehydrogenase which catalyzes the second step of the proline degradation pathway, converting pyrroline-5-carboxylate to glutamate. Deficiency of this enzyme is associated with type II hyperprolinemia, an autosomal recessive disorder characterized by accumulation of delta-1-pyrroline-5-carboxylate (P5C) and proline. Alternatively spliced transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq, Jun 2009]
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UniProt Comments for ALDH4A1
ALDH4A1: Irreversible conversion of delta-1-pyrroline-5- carboxylate (P5C), derived either from proline or ornithine, to glutamate. This is a necessary step in the pathway interconnecting the urea and tricarboxylic acid cycles. The preferred substrate is glutamic gamma-semialdehyde, other substrates include succinic, glutaric and adipic semialdehydes. Defects in ALDH4A1 are the cause of hyperprolinemia type 2 (HP-2). HP-2 is characterized by the accumulation of delta-1-pyrroline-5-carboxylate (P5C) and proline. The disorder may be causally related to neurologic manifestations, including seizures and mental retardation. Belongs to the aldehyde dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 1.2.1.88; Amino Acid Metabolism - arginine and proline; Amino Acid Metabolism - alanine, aspartate and glutamate; Mitochondrial; Oxidoreductase

Chromosomal Location of Human Ortholog: 1p36

Cellular Component: mitochondrial matrix

Molecular Function: identical protein binding; aldehyde dehydrogenase (NAD) activity; electron carrier activity; 1-pyrroline-5-carboxylate dehydrogenase activity

Biological Process: 4-hydroxyproline catabolic process; proline catabolic process; proline biosynthetic process; glutamate biosynthetic process; proline catabolic process to glutamate; proline metabolic process

Disease: Hyperprolinemia, Type Ii
Research Articles on ALDH4A1
1. Missense mutation in ALDH4A1 is associated with succinic semialdehyde dehydrogenase deficiency and severe intellectual disability.
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Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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