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FLASH GENE

Symbol

CLPB

contributors: mct - updated : 25-02-2015

HGNC name	ClpB caseinolytic peptidase B homolog (E. coli)
HGNC id	30664

FLASH GENE DNA RNA EXPRESSION PROTEIN DISORDER ANIMAL & CELL MODELS

Corresponding disease	MGCA7 3-methylglutaconic aciduria, type VII
Location	11q13.4      Physical location : 72.003.471 - 72.145.568
Synonym name	suppressor of potassium transport defect 3
	caseinolytic peptidase B protein homolog
Synonym symbol(s)	SKD3, HSP78, FLJ13152, HSP104

DNA

TYPE	functioning gene
STRUCTURE	142.26 kb     17 Exon(s)

MAPPING

cloned

Y

linked

N

status

provisional

RNA

TRANSCRIPTS	type	messenger

identification nb exons type bp product Protein kDa AA specific expression Year Pubmed NM_001258393 15 - 3095 NP_001245322 - 648 - 2006 16834329 NM_001258392 16 - 3182 NP_001245321 - 677 - 2006 16834329 NM_030813 17 - 3272 NP_110440 - 707 - 2006 16834329 NM_001258394 18 - 3379 NP_001245323 - 662 - 2006 16834329

EXPRESSION

Type

expressed in	(based on citations)
organ(s)	System Organ level 1 Organ level 2 Organ level 3 Organ level 4 Level Pubmed Species Stage Rna symbol Digestive intestine large intestine colon       stomach         Endocrine adrenal gland         Nervous brain       highly Homo sapiens Adult Reproductive female system breast mammary gland       male system testis       Skin/Tegument skin         Urinary kidney         Visual eye

cells

System Cell Pubmed Species Stage Rna symbol Blood/Hematopoietic granulocyte Homo sapiens

cell lineage
cell lines
fluid/secretion	blood, lymph

at STAGE

PROTEIN

PHYSICAL PROPERTIES

STRUCTURE

motifs/domains	four ANK repeats in the N-terminal part of the protein might have evolved to ensure a more elaborate or more refined substrate recognition, to mediate the interaction with as yet unknown protein partners, or even to support a putative chaperone function
	first 92 AAs are predicted to encode a mitochondrial targeting sequence
	two AAA+ modules, that bind and hydrolyze ATP and construct the core of the hexameric ring
	a specific C-terminal D2 domain, which is typical for AAA+ proteins involved in polypeptide chain threading through the hexamer central channel

mono polymer

homomer , hexamer

HOMOLOGY

interspecies	homolog to murine Clpb
	ortholog to rattus Skd3

Homologene

FAMILY	clpA/clpB family
	AAA+ protein family

CATEGORY

chaperone/stress , enzyme , regulatory

SUBCELLULAR LOCALIZATION	intracellular
	intracellular,cytoplasm,organelle,mitochondria

basic FUNCTION	may function as a regulatory ATPase and be related to secretion/protein trafficking process
	protect cells from the devastating effects of protein inactivation and aggregation that arise after extreme heat stress
	can passively thread soluble denatured proteins
	CLPB hexamers remain associated during several ATP hydrolysis events required to partially or completely translocate substrates through the protein central channel, and CLPB dynamics is tuned by DnaK and substrate proteins
	its function might be related to apoptosis
	key role of CLPB in regulating protein folding inside the mitochondrial matrix

CELLULAR PROCESS

PHYSIOLOGICAL PROCESS

exocytosis transport , cellular trafficking transport

PATHWAY

metabolism

signaling

a component

forms a hexameric, ring-shaped complex that functions as a tightly regulated, ATP-powered molecular disaggregation machine

INTERACTION

DNA

RNA

small molecule	nucleotide,
	ATP binding

protein

interacts biochemically with ATP2A2, known to be involved in apoptotic processes in severe congenital neutropenia

cell & other

REGULATION

ASSOCIATED DISORDERS

corresponding disease(s)

MGCA7

Susceptibility

Variant & Polymorphism

Candidate gene

Marker

Therapy target

ANIMAL & CELL MODELS

suppression of clpb in zebrafish embryos induced a central nervous system phenotype that was consistent with cerebellar and cerebral atrophy